Pathogenesis

Rheumatoid arthritis
INTRODUCTION:
-Chronic, Systemic, Inflammatory, Autoimmune disease.
-Primary target - synovial tissues.
-Persistent inflammatory synovitis.
-Cartilage damage and bone erosions - changes in joint
integrity.
-Variable course
-Unchecked disease - substantial disability and
premature death.

-Prevalence - 0.8% (range 0.3 to 2.1%).

-RA is seen throughout the world and affects all races.
-Women are affected three times more than men.
-Sex differences diminish in the older age group.
-The prevalence increases with age.
-The onset is most frequent during the fourth and fifth
decades of life.
Clinical features

-Prodromal symptoms
ETIOLOGY
-Fatigue, anorexia, weakness
-Unknown.
-Vague musculoskeletal symptoms
-Genetic predisposition: severe RA four times more
st -Fever
common in 1 degree relatives
-Lymphadenopathy
-HLA-DR4: Manifestation of the response to an infectious
agent in a genetically susceptible host. -These symptoms precede in some
-Mycoplasma, CMV, EBV: A triggering event, possibly
autoimmune or infectious, initiates joint inflammation. -Symmetrical deforming polyarthritis:
-Complex interactions. -affects synovial lining of joints, bursae and
tendons
-more then just joint disease
Pathology -Presentation
-Pathologic hallmarks of RA are: -Variable
-Synovial inflammation and proliferation -Gradual or acute/subacute
-Focal bone erosions -Palindromic
-Thinning of articular cartilage -Monoarticular
-Symmetrical, diffuse small joint involvement
-Chronic inflammation leads to synovial lining
hyperplasia and the formation of pannus -(a thickened Joint involvement
cellular membrane of granulation-reactive fibrovascular -Morning stiffness
tissue that invades underlying cartilage & bone) -> 1 hour
-Improves with activity
-Adjacent bone marrow involvement
-Small joints of hands and feet
-Generalized osteoporosis
-Wrist
-Elbows

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 -Shoulders Shoulders
-Knees and ankles -Commonly affected
-May mimic frozen shoulder
Hands and wrists -Later global stiffening may occur
-Impact – severe -disability
-Fingers – swollen, painful and stiff
-Deformities Feet
-Swan neck deformity ( fixed -Earliest manifestation – painful swelling of
hyperextension) MTP joints
-Boutonniere’s deformity (fixed flexion -Foot – broader, hammer toe deformity,
deformity) plantar subluxation of metatarsal heads
-Ulnar deviation -Eversion at the hind foot( subtalar joint)
-Z thumb -Flat medial arch- flat foot
-May cause carpal tunnel syndrome -Ulcers may develop at exposed metatarsal
heads
Swan neck deformity
Knees
-Synovitis
-Knee effusions
-Long standing – fixed flexion deformities
-Atrophy of the muscles of thigh
-Debilitating

Boutonniere’s deformity Cervical spine

-Stiffness of neck – muscular
-Rheumatoid synovitis may affect the cervical
vertebrae
-May cause atlanto-axial subluxation;
emergency
-quadriparesis
-Bowel bladder symptoms

Extraarrticular manifestations(V R S)
-Variety of extra articular features
-40% have them (~15% severe)
-These occur in those with high titres of
Rheumatoid factor. (autoantibodies to the Fc
component of IgG)

1. Rheumatoid nodules
-20 -30%
-Found on periarticular structures, extensor surfaces,
areas subjected to mechanical pressure
-Rarely – meninges and pleura
-Variable size and consistency
-Asymptomatic but can rupture- infections
-Methoteraxate treatment – may increase

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 6. Others
-Pericarditis, pericardial effusion
-Entrapment neuropathy
-Scleromalaciaperforans, episcleritis
-Morrant Baker’s cyst

2. Rheumatoid vasculitis
-Can affect any organ
-Can cause-
-Polyneuropathy, mononeuritis multiplex
-Cutaneous ulceration, dermal necrosis, digital
gangerene Sjogren’s syndrome
-Visceral infarction -Episcleritis
-Renal vasculitis - rare -Scleromalacia
-Kerato-conjunctivitis sicca
-Features of rheumatoid arthritis

3. Pleuro pulmonary involvement

-Common in men
-Pleural disease, interstitial fibrosis, pleuropulmonary Laboratory Investigations
nodules, pneumonitis, arteritis. -Anemia – Normocytic normochromic
-pleural fluid – low glucose -ESR & CRP – raised in proportion to disease activity
-Caplan’s syndrome - pneumoconiosis with diffuse -Rheumatoid Factor (RF)
nodular fibrotic process -Serum ANTI-CCP antibodies
-same sensitivity as serum RF
-specificity approaches 95%
-Positive anti-CCP antibodies useful for
distinguishing RA from other forms of arthritis
-Some patients with RA are positive for RF but negative
for anti-CCP and visa versa
-RF or anti-CCP antibodies also has prognostic
significance, with anti-CCP antibodies showing the most
value for predicting worse outcomes

Rheumatoid factor
-45% positive in first 6 months
4. Felty’s syndrome
-85% positive with established disease
-Chronic RA, neutropenia, splenomegaly
-Not specific for RA, high titer early is a bad sign
-Occ. Thrombocytopenia and anemia
-found in 75–80% of patients with RA
-Increased frequency of infections
-negative result does not exclude the presence of this
5. Osteoporosis disease
-Secondary to rheumatoid involvement -also found in other connective tissue diseases & chronic
-May be aggravated by steroid therapy; Osteopenia infections
involves juxtaarticular bone and long bones distant from -may also be detected in 1–5% of the healthy population
involved joints

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Imaging ACR/EULAR classification criteria 2010
a. X-ray: Joint involvement Score
-erosion of cartilage & bone 1 large joint (S/E/H/K/A) 0
2-10 large joints 1
1-3 small joints (MCP/PIP/Thumb IP/MTP/wrist) 2
4-10 small joints 3
>10 joints (at least 1 small joint) 5

Acute phase reactants

b. MRI: Normal CRP and ESR 0
-more sensitive than plain x-ray Abnormal CRP or ESR 1
-detects bone erosion earlier
-bone marrow edema (predictor of later development of Serology
erosive disease) Neg RF & Neg anti-CCP 0
-to quantify hypertrophic synovial tissue Low + RF & Low + anti-CCP (<3 titer) 2
High + RF & High + anti-CCP (>3 titer) 3
c. USG:
-estimates the degree of inflammation and volume of the Duration
inflammed tissue < 6 weeks 0
-can also be used to assess MTP joints >6 weeks 1

Total score 0-10. > 6 RA

Synovial Fluid Analysis

-Reflects an inflammatory state
3
-WBC counts – 5000 and 50,000 WBC/
-As compared to <2000 WBC/ for a non-
inflammatory OA
-Overwhelming cell type is neutrophil
-Also contains RF and anti-CCP antibodies and
immune complexes, as well as by-products of
complement activation
-Analysis of synovial fluid is most useful for:
-Confirming an inflammatory arthritis
-Excluding osteoarthritis, infection or a crystal-
induced arthritis

Diagnostic criteria
ACR 1987 (4/7 necessary)
1. Morning stiffness (> 1 hour)
2. Arthritis of at least 3 areas (> 6 weeks)
3. Arthritis of hand joints
4. Symmetrical arthritis
5. Rheumatoid nodules
6. Serum rheumatoid factor
7. Radiographic changes
Duration of >6 weeks

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Diagnosis Management
-Maybe delayed due to non specific initial symptoms - Remission of symptoms involving the joints.
-Haematology:Hb, wcc, plts, ESR - A return of full function.
-Biochemistry:U+E, LFT, CRP - The maintenance of remission with DMARD therapy.
-Immunology: RhF, ANA
-Microbiology: viral titres
-Radiology: XR, bone scan, MRI
THERAPEUTIC STRATEGIES
-Early diagnosis and treatment .
Differential Diagnosis -The use of disease-modifying antirheumatic drugs
-Postviral arthritis (DMARDs) in combination is highly effective.
-Rubella -The use of agents that target cytokines, such as TNFa
-Hepatitis B and interleukin-1, is an effective strategy.
-Parvovirus -An analysis of important coexisting illnesses
-Chikungunya (particularly cardiovascular disease and osteoporosis).
-Seronegative Spondyloarthropathies
-Bacterial endocarditis
-Rheumatic fever TREATMENT
-Sarcoidosis a. Non steroid anti-inflammatory drugs ( NSAIDs ) :
-Reactive arthritis -Aspirin, Indomethacin, Propionic acid compounds ,
-Psoriatic arthritis Diclofenac, Piroxicam.
-Systemic lupus erythematosus -COX -2 inhibitors – Celecoxib, Roficoxib.
-Primary Sjögren's syndrome b. Corticosteroids:
-Chronic tophus gout c. Disease modifying anti rheumatic drugs ( DMARDs ) :
-Calcium pyrophosphate disease Synthetic : Methotrexate, chloroquine, penicillamine,
-Polymyalgia Rheumatica gold, sulphasalazine , azathioprine, cyclophosphamide,
-Osteoarthritis (erosive) cyclosporine, levamisole, minocycline, leflunomide.
Biological : Infliximab , Adalimumab, Etanercept,
Anakinra, Rituximab, Abatacept, Toclizumab
Rheumatoid arthritis functional grading
Grade I
-No handicap
-Can carry on all daily activities
Grade II
-Moderate restriction of activities but
independent
Grade III
-Marked restriction of activities mostly limited
to self care
-Needs assistance
Grade IV
-Bed or chair bound
-Incapacitated and dependent

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NSAIDS INFLIXIMAB :
-Not been shown to slow the progression of the disease. -Chimeric IgG1 anti–TNFaantibody
-Should be used together with DMARDs.
ADALIMUMAB
COX-2 inhibitors : -Recombinant human IgG1 monoclonal antibody
-Celecoxib : 100-200 mg bd
-Roficoxib : 12.5- 50 mg od
-Efficacy – same.
Surgery
-In patients with severely damaged joints
-Arthroplasty and joint replacement surgeries
CORTICOSTEROIDS: -Reconstuctive hand surgery
-Decrease the progression of rheumatoid arthritis as
detected radiographically.
-Side effects :
Prognosis
- Thinning of the skin, cataracts, osteoporosis,
-Life expectancy reduced by
gastritis, hypertension, hyperlipidemia.
-7 years in men
-3 years in women
-Severe morbidity
SYNTHETIC DMARDS -sudden onset do better than gradual
-Sustained suppression of inflammation. -early knee involvement bad
-Retard or halt the progression of disease. -Bad RA has a worse prognosis than IHD or Hodgkins
-Decrease radiographic progression.

METHOTREXATE :
-Most likely to induce a long-term response.
-Most often selected for initial therapy.
-Demonstrated efficacy and durability, acceptable
toxicity, and low cost.
-Lower mortality.

LEFLUNOMIDE :
-Efficacy similar to moderate dose mtx.
-MOA :
-Immunomodulator.
-Inhibits denovo synthesis of pyrimidines- anti
proliferative effect.
-Dose :
Loading dose of 100mg od for 3 days
f/b 20 mg/day p/o.
-S/E :Elevation of liver enzymes, diarrhoea

BIOLOGICAL DMARDS:

ETANERCEPT :
-A recombinant soluble TNF-receptor fusion protein.
-Binds to both TNFaand TNFb.

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 -Multi-system
Systemic Lupus Erythematosus
-Inflammatory
Introduction: Connective Tissue Disease -Difficult to diagnose
-When studying rheumatic disease it can be helpful to -Treated with steroids and immunosuppressive drugs
classify diseases into categories
-One of those groups is Connective Tissue Diseases
What is the consequence of having a CTD?
-Difficult diagnosis
How do CTDs fit in? -Relatively uncommon diseases
Rheumatic Diseases (Rheumatology) -Multi-system
-Degenerative arthritis -Significant morbidity and mortality
-Rheumatoid arthritis -Treatment
-Seronegative arthritis -Psychosocial implications
-Infectious arthritis
-Crystal Arthritis
-Connective Tissue Diseases Clinical Clues
-Vasculitis - Inflammatory: fever, constitutional symptoms
-Involvement of skin
-Associated arthritis
History -Raynaud’s phenomenon
-In 1942, the term “diffuse collagen disease” was used to
describe systemic lupus erythematosus (SLE) and
scleroderma
-Later the term “collagen- vascular disease” was
introduced to describe vasculitis SYSTEMIC LUPUS ERYTHEMATOSUS
-Then in 1952, the term “connective tissue disease” was
Definition
introduced
SLE is an autoimmune disease in which organs, tissues,
-This term replaced the other terms
and cells undergoes damage mediated by tissue-binding
autoantibodies and immune complexes

What does this include? -Autoimmune rheumatic disease

-Systemic Lupus Erythematosus -Commonly effect the skin & musculoskeletal systems
-Scleroderma -but can affect every organ
-Sjogren’s Syndrome -Kidney - Central Nervous System
-Inflammatory Myopathy -Heart - Lungs
-Polymyositis
-Diversity of presenting symptoms complicates the
-Dermatomyositis
diagnosis
-Mixed Connective Tissue Disease
-American College of Rheumatology (ARA) published
-Antiphospholipid Antibody Syndrome
guidelines for disease classification(1971, revised 1982 &
-*Rheumatoid Arthritis
1997)
-Patients need to fulfil 4 of the 11 criteria to reach a
diagnosis of SLE
Common Features
-Uncommon
-Autoimmune

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What Causes SLE Type III Hypersensitivity Reaction (Immune Complex-
-Cause is unknown Mediated)
-Possible factors -Hyperactivity of T and B-cells
-Genetic -Formation of immune complexes
-Environmental -Clearance via the complement system
-Type III Hypersensitivity Reaction
What Happens? -Tissue damage
-Loss of self-non-self recognition
-Diversification of antigen-driven responses Key Concepts
-Hyperactivity of T & B Cells * Self-non-self *
-Formation of immune complexes Specificity
-Type III hypersensitivity reaction Memory

Loss of T-cell Tolerance EPIDEMIOLOGY

-T-Cells found in healthy & diseased patients -Incidence varies with racial origin (1:250 in black
-Loss of self-non-self-recognition allows T-Cells to react American women)
with self-antigen. -Sex ratio in childhood SLE varies to that of adults (more
boys than girls in paeds, 9 times as common in women as
men)
-? Genetic component (related to incidence/area) “Twins
study”

Clinical Features (1)

-Classical photosensitive butterfly rash
-Vasculitic lesions of the hands & feet (or Raynard’s
phenomenon
-Profound tiredness
-Arthralgia or even arthritis
-? Alopecia
-Oral ulcers less common in children than in adults

Pathogenesis
once T-cell tolerance is lost

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Raynaud’s Phenomenon -Psychosis
-Reversible skin color change (white to blue to red) -Seizures
-Induced by cold or stress
-Episodes of transient digital ischemia
-Represent vasoconstriction of digital vessels
Investigations
-Serological for end organ dysfunction and
immunological tests (looking for the autoantibody profile
of lupus)
-FBC (anaemia,lymphopenia, thrombocytopaenia)
-Raised ESR
-Low to normal complement levels
-Raised ANA

-Lumber puncture
-Can be primary (no associated disease)
-Electroencephalogram (EEG)
-Or secondary (there is an associated disease)
-Magnetic resonance imaging (MRI)
-Often, the associated disease is a CTD
-Computed tomography (CT)
-Skin biopsy

Other Clinical Features

-Pericarditis
Lupus Nephritis
-Pleuritis
-Or “Lupus glomerulonephritis”
-Pulmonary vasculitis or haemorrhage
-? Developed by approx 1/3 of lupus patients (50% in
-Some present initially with renal involvement
adults within 1 yr of diagnosis)
-cerebral involvement (Lupus cerebritis -most common
-15 to 20% will require RRT (Pollak & Pirani 1997)
cognitive dysfunction, headaches & depression)
-Proteinuria (45 to 65% will develop nephrotic syndrome
Serositis
-associated fluid retention, weight gain, oedema
-Microscopic Haematuria increases to 90% of patients
(Cameron, 1999)

-Mild to severe renal compilcations

-Commonly UTI’s
-Medications may confuse renal signs ?
-BUN, creatinine, serum albumin, U&E’s
-USS, or renal biopsy (for confirmation)

Blood Disorder
Renal Disease
-Hemolytic anemia
-Leukopenia
-Thrombocytopenia

Neurological Disease
-Other CNS symptoms chorea, seizures, CVA, cranial
neuropathy + visual loss
-Functional psychosis, personality disorder, & memory
alteration

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Diagnosis: ARA Classification for SLE A = ANA positive
I = immunological disorder
1. Malar rash N = neurological disease
2. Discoid rash
3.Photosensitivity
4. Oral ulcers
5. Arthritis Therapies for SLE
6. Serositis: st
1 Therapies
-pleuritis
-Cytotoxic drugs
-pericarditis
7. Renal disorder Current Therapies
-Proteinuria >0.5g/24h or >3g persistently or -Immunosuppressions
-cellular casts -Cyclophosphamide
8. Neurological disorder -Mycophenolate Mofetil
-Seizures
-psycosis (having excluded other causes) Future Therapies
9. Haemotological disorder -Hormonal modulation
-Haemolytic anaemia or -Cytokine inhibition
9
-Leucopenia or <4.0 x 10 /l on 2 0r more
occasions
9
-Lymphopenia or <1.5 x 10 /l on 2 or more Therapeutic choices depends upon
occasions -If life threatening or likely to cause organ damage
9
-Thrombocytopenia <100 x 10 /l -If manifestations are potentially reversible
10. Immunolgical disorders - Best approach to prevent complications of disease and
-Raised antinative DNA (antibodies to genetic treatment
material in cells)
-anti-Sm antibody (Sm is a protein found in the
cell nucleus)
-+ve finding of antiphospholipid antibodies Drug Therapy
based on -Anti-inflammatory drugs
-abnormal IgG or IgM -Corticosteroids
-anticardiolipin levels or -Anti-malarial drugs
-+ve test for lupus anticoagulant or -Immunosuppressive RX
-False-positive serological test for syphilis -Symptomatic therapy?
present for at least 6 months
11. Antinuclear antibody in raised titre (autoantibodies
to cell nuclei) Anti-inflammatory Drugs
-Reduce inflammation & pain
SOAP BRAIN MD
-Particularly effective for low-grade fever, fatigue,
S = serositis
arthritis & pleurisy
O = oral ulcers
-NSAID’s = salicylates (asprin, ibuprofen)
A = arthritis
P = photosensitivity

M = malar rash Corticosteroids

D = discoid rash -For more active disease with arthritis, pericarditis,
pleuritis or serositis.
B = blood disorder
-IV or orally
R = renal disease

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-Maintenance in UK of oral enteric coated pred. Scleroderma
-Acute flares IV methylpred (for life or organ threatening
disease) Case History
-(don’t forget side effects, osteoporosis, atherosclerosis, -35 years female
acute psychosis, myopathy) -No formal education

Anti-malarial Drugs Presenting complaints

-Particularly effective against arthritis, skin rashes & 1. Joint pain 7 mths
mouth ulcers 2. Tightening of skin 6 mths
-Hydroxychlorquine may take 4-6 months before a 3. Bluish discoloration of hands on cold exposure 3 mths
benefit is seen 4. Difficulty in swallowing 3 mths
-Side effects = ? Harm foetus, gastric symptoms,rash,
-Past and family history unremarkable
darkening of skin, muscle weakness, retinal damage
-Married, husband farmer, non consanguineous
-Eye exam before then 6 monthly
marriage, one daughter 4 yrs old, menses regular

Immunosuppressive Agents
Examination
-Which agent? When ? + still controversial
-Pulse 84 beats per min
-Azathioprine
-BP 110/70 mmHg
-Cyclophosphamide in severe cases, (especially in lupus
-RR 20 breaths per min
nephritis & cerebritis)
-Temperature 99 F
- ? IV cyclophos monthly for 6 months, followed by
-Pallor present
infusions 2-3 monthly for 2 years (Thomas et al, 2000)
-OTHER SYSTEMIC EXAMINATION WITHIN NORMAL
-Methotrexate (Ravelli et al, 1998)
LIMITS
- (sperm or egg storage!!)
-Thickening and tightening of skin of both hands proximal
and distal parts, face, neck, abdomen, back, and proximal
Plasmapheresis and distal part of the lower limb
-In conjunction with cyclophos
-Role in the management of severe lupus remains
controversial
-? “Life-threatening situations” it may `buy time

Systemic Lupus Erythematosus

-Drug-induced lupus -Multiple hypopigmented macular lesions on upper chest
-Neonatal lupus and front of neck ranging in size from 5mm to 10mm
diameter, discrete and confluent lesions
-Reduced opening of mouth, with reduced size of mouth
opening aperture

Investigations
-Hb 8.7 gm/dl
3
-TLC 10900/ mm N80 L20
3
-Platelets 167000/mm

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-RBS 76 mg/dl SYSTEMIC SCLEROSIS
-Urea 20 mg/dl
-Creatinine 0.8 mg/dl Multisystem disorder of unknown etiology characterized
st
-ESR 66 mm 1 hour clinically by thickening of skin caused by accumulation of
connective tissue and by structural and functional
-Urine: protein –trace, WBC 0-2 abnormalities of visceral organs, including the
-24 hour urinary protein 0.55 gm gastrointestinal tract, lungs, heart and kidneys
-Creatine Kinase total 2425 U/L
-CXR , ECG -Within Normal limits
-UGI endoscopy –could not be done
Epidemiology
-RA factor < 8 IU/ml -Annual Incidence 19 per million
-ANA 281.77 IU/ml ( >55 positive) -Prevalence 19-75 per 100,000 persons
-Anti dsDNA 32.53 IU/ml (>40 positive) -Women > Men (3:1)
rd th
-Skin biopsy showed mild perivascular chronic -3 to 5 decade
inflammatory infiltrate -African American, Choctaw Native Americans in
Oklahoma
-Childbearing years more common

Investigations would like to-do

-Anti-topoisomerase-1 antibody
-Anticentromere antibody PATHOGENESIS

Diagnosis
SYSTEMIC SCLEROSIS (Diffuse Cutaneous)

Differentials
-Overlap syndrome
-CREST syndrome

Treatment
-Vaseline Lotion – Skin Care
-Nifedipine/ Pentoxiphylline
-NSAID ( Nimesulide)

CLINICAL MANIFESTATIONS
Discharge SKIN
-Explained the disease process 1. Edematous phase
-Advised for follow up and continuation of prescribed 2. Indurative phase
drugs 3. Atrophic phase

Others
Raynaurds
Hypopigmentation
Hyperpigmentation

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 Telengiectasia PREGNANCY ASSOCIATED
Typical facial appearance -Preterm birth
-Flare up-renal crisis
FACIAL APPEARANCE -Improve-Raynaud’s, Worsen-Esophageal reflux
-Steen et al, 91 pts, 10 year follow up- No increase in
incidence of miscarriage

CREST syndrome
C – calcinosis
R – raynaurd’s
E- esophageal dysmotility
GASTROINTESTINAL TRACT S- sclerodactyly
-Dysphagia T- telengectasia
-Hypomotility
-Reflux
-Fibrotic strictures DIAGNOSTIC CRITERIA
-Malabsorption MAJOR
-Pseudoobstruction -Presence of Sclerodermatous involvement proximal to
-Primary biliary cirrhosis the digits, affecting proximal portions of the extremities,
-Pancreatic exocrine insufficiency face, neck or trunk
MINOR
LUNGS
1. Sclerodactyly
-Pulmonary arterial hypertension
2. Digital pitting scars or Tissue loss of volar pads of
-Pulmonary fibrosis
fingertips
HEART 3. Bibasilar pulmonary fibrosis
-Restrictive cardiomyopathy
American college of rheumatology
-Pericardial effusion
Diagnosis 1 major or ≥2 minor
-Conduction defect
Senitivity 97 % and Specificity 98 %
-Infarction
-Ventricular tachycardia
-Pericarditis
Classification of scleroderma/ systemic sclerosis and
KIDNEYS scleroderma like disorders
-Proteinuria -Systemic sclerosis
-Azotemia -Localised scleroderma
-Arterial hypertension -Chemically induced scleroderma like disorders
-Other scleroderma like disorders
JOINT AND MUSCLE
-Arthritis
-Arthralgia
-Proximal muscle weakness Systemic sclerosis
-Limited cutaneous disease
NEUROLOGICAL -Diffuse cutaneous disease
-Peripheral neuropathy -Sine scleroderma
-Trigeminal neuralgia -Undifferentiated connective tissue disease
-Overlap syndrome

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Localized scleroderma Treatment
-Morphea -D-penicillamine
-Linear scleroderma -Colchicine
-En coup de sabre -IFN-γ
-IFN-α
-Recombinant human relaxin

Chemically induced scleroderma like disorders Other agents

-Toxic-oil syndrome -Chlorambucil
-Vinyl chloride-induced disease -Cyclosporine
-Bleomycin-induced fibrosis -5-fluorouarcil
-Pentazocine-induced fibrosis -Thalidomide
-Epoxy- and aromatic hydrocarbons-induced fibrosis -Etanercept
-Eosinophilia-myalgia syndrome
For arthritis/ arthralgia
-NSAIDS
-Low dose steroid
Other scleroderma like disorders
-Scleredema adultorum of Buschke For Raynaud’s phenomenon
-Scleromyxedema -Prazocin, Reserpine
-Chronic graft-vs-host disease -Diltiazem, Nifedipine
-Eosinophilic fasciitis -Ketanserin, Fluoxetine
-Digital sclerosis in diabetes -Pentoxiphylline
-Primary amyloidosis and amyloidosis associated with -Alprostadil, Bosentan
multiple myeloma
Others
-ACE-inhibitor for Systemic Hypertension
-Antibiotics
Lab findings
-Metoclopromide-Esophageal dysmotility
-Raised ESR
-Anemia Prognosis
-Rheumatoid factor 25% Survival→ 5 year 10 year
-ANAs Limited 90% 75%
-antitopoisomerase 1 (scl 70) Diffuse 70% 55%
-antinucleolar -Death mostly due to pulmonary, cardiac and renal
-anticentromere involvement

Treatment modalities
-Supportive measures
-Physiotherpy
-Skin care
-Psychological guidance
-Pharmacological treatment

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 Risk Factors for Gout
Crystal-induced Arthropathies
-Obesity, metabolic syndrome
Introduction
-Ethanol (particularly beer and spirits)
-Gout and pseudogout are the 2 most common crystal-
-Diuretics- thiazides
induced arthropathies.
-Low dose aspirin(< 1g/d)
-Gout is caused by monosodium urate monohydrate
-Fructose ingestion
crystals
-Excessive purine ingestion (meat & sea food)
-Pseudogout is caused by calcium pyrophosphate crystals
and is more accurately termed calcium pyrophosphate Underexcretors (80%)
disease. -Male gender
-Postmenopausal females
-Obesity, metabolic synd.
History -Ethanol
-Ancient disease: “the king of diseases and the disease of -Renal insufficiency
kings” -Plumbism
- first identified by the Egyptians -Medications (see separate)
-5thcentury BC: Hippocrates referred to gout as -Dehydration/low flow
“unwalkable disease” and noted links between gout & -Filipino ancestry
lifestyle, demographics & other variables -Fructose ingestion
-Uromodulin kidney dis.
-Crystals from tophi were first described during the 18th
and 19th centuries. Overproducers (20%)
th
- In the mid 20 century the role of excess urate -Ethanol
production and impaired excretion in the pathogenesis of -High cell turnover states (psoriasis, myeloprolif.
hyperuricemia were reported. disorders)
-Finally, McCarty and Hollander showed that crystals -Excessive purine ingestion
from the synovial fluid of patients with gout were -PRPP overactivity (x-linked)
composed of MSU -HGPRT underactivity (x-linked)
-Beta aldolase deficiency
-Sarcoidosis
-B12 deficiency
Definition -Down syndrome
-Gout is a syndrome caused by the inflammatory -Glycogen storage dis. 3, 5, 7
response to tissue deposition of monosodium urate -Fever, post-op state
crystals (MSU).
-It is associated with hyperuricemia

Hyperuricemia and Gout

Epidemiology -Hyperuricemia (>7.0 mg/dl) in 5% - 8% of male
-Rates of gout in USA increased during the 1990s— population.
especially for men older than 75 years in whom rates -Most (about ⅔) are forever asymptomatic.
nearly doubled from 2.1% in 1990 to 4.1% in 1999. -80% of gouty patients have uric acid < 9 mg/dl.
-In USA 2.6 million in 2005, projected to increase to 3.6 -Above 10 mg/dl, risk rises rapidly.
million in 2025 -Gout is the most common cause of monarthritis in
-The prevalence of gout is much higher in men than in middle-aged and elderly men (8% yearly prevalence).
women and rises with age.

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-Dairy consumption, coffee and vitamin C are protective. 1.Asymptomatic hyperuricemia
-Estrogen protective (suppresses URAT1, the proximal -Clinical laboratories define hyperuricemia as > 8-8.5
renal tubule epithelial cell anion exchanger). mg/dl
-Urate-lowering therapy can trigger attacks in the early -In physiologic terms , any levels > 6.8 mg/dl because it
period after its initiation, presumably as a result of exceeds the soluble concentration of MSU in body fluid
mobilization of bodily urate stores. -Incidence of gout increases with age as well as degree of
hyperuricemia
-However vast majority of people with hyperuricemia
never develop symptoms associated with uric acid excess
Drugs Associated with Hyperuricemia
-Diuretics (loop and thiazide types)
-Low-dose aspirin
-Cyclosporine, tacrolimus
-Ethanol
-Ethambutol
-Pyrazinamide
-Ritonavir, darunavir, didanosine
-Levodopa .
-Nicotinic acid, niacin
-Pancreatic enzymes 2. Acute intermittent gout
-Rituximab
-Decades of untreated, chronic hyperuricemia increases
-Basiliximab
body urate stores, advancing the severity of the disease:
-Teriparatide
-Flares last longer
-Filgrastim
-Flares occur more often
-Sildenafil
-Intercritical segments (flare free periods)
-Diazoxide
decrease
-Cytotoxic chemotherapy
-Persistent pain and stiffness occur

A typical attack of gout:

Classifications:
-Rapid development of warmth, swelling, erythema and
a. Primary Gout – 90% pain in the affected joint
-Overproduction -Pain escalates to most intense level in 8-12 hrs
-Under excretion -Initial attack is usually monoarticular and >50% cases
st
involve the 1 MTP joint.
b. Secondary Gout – 10% -Other joints involved in early stage: midfoot, ankles,
heels and knees.
-Less commonly wrists , fingers and elbows.
Progression
1.Asymptomatic hyperuricemia
2.Acute gouty arthritis
3.Intercritical Gout
4.Chronic / Tophaceous Gout

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Acute attacks can be precipitated: Tophi are frequently seen
-Increased alcohol consumption (especially beer) -Helix of the ear
-Trauma -Over the olecranon processes
-Use of diuretics -On the Achilles tendons
-Dehydration -Within and around the toe or finger joints
-Cyclosporine -Around the knees, and within the pre-patellar bursae
-Diet (organ meat, shellfish) -Spinal involvement can lead to nerve root or cord
-Drugs that can lead to sudden changes (increase or compression and might also mimic epidural infection
decrease) in urate levels, such as hypouricemic agents -Median nerve, which leads to carpal tunnel syndrome
-Rare locations: eyes, breast, vocal cords, heart, and
colon
3. Intercritical Gout
-Previously involved joints are virtually free of symptoms
-Despite this, MSU crystals often can be identified in the
synovial fluid of previously inflamed joints which reflect
ongoing subclinical inflammation

Differential dx:
1. Crystal-induced arthritis:
-CPPD
-Hydroxyapatite
-Calcium oxalate
2. Infectious arthritis
DIAGNOSIS
3. Systemic diseases:
*Physical examination and medical history
-Psoriatic arthritis
*Measuring blood uric acid levels
-RA
*Examination and analysis of synovial fluid
-Reactive arthritis
*Radiography

Physical examination and medical history

-patient’s description of symptoms

Gout is more likely if:

4. Advanced gout
-Arthritis first appears in the big toe than if it first
-It is characterized by chronic destructive polyarticular appears elsewhere
involvement with low-grade joint inflammation, joint -Onset of symptoms (pain and swelling) takes days or
deformity, and tophi weeks .symptoms which take hours to develop probably
-Tophaceous gout develops within 5 years of onset of indicate a disorder other than gout.
gout in 30% of untreated patients -Abnormal enlargements in joints that had been affected
-Tophi are painless and rarely become infected but by previous injury or osteoarthritis are possible signs of
function and health-related QoL can be severely affected gout
with chronic gout

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Measuring blood uric acid levels The Three Phases of Gout Treatment
-Uric acid levels in the blood during an attack of gout can -Treat acute attack
lie within or below the normal range -Prevent new attacks
or -Reduce uric acid level (sometimes)
-Hyperuricemia may be prevalent in population and
doesn’t necessarily indicate gout.

Phase 1 – Termination
-NSAID
Synovial Fluid in Gout -Colchcine
-May be cloudy or clear. -Intra-articular steroids
-Inspect for tophaceous deposits. -Systemic steroids
-WBC – 2000 – 50,000 or more… -IL-1 inhibitors
-Glucose normal.
-Between attacks, may have free crystals.
-Don’t forget to culture it. NSAIDs
-Treatment of choice in otherwise healthy patient.
-Avoid in renal insufficiency and in peptic ulcer disease.
-Avoid salicylates (these cause swings in serum uric acid).
Send Synovial Fluid for:
-Cell count Lavendar top Intra-Articular Steroids
-Crystals Green top -One or a few joints.
-Culture Red top, no preserv. -Not useful for polyarticular or soft-tissue gout.
-Glucose Red top -Make sure infection not present.
-Protein, pH, complement – not helpful
Oral Colchicine
-1.2 mg followed by 0.6 mg 2 hrs later.
-Loading dose same in renal insufficiency.
Reasons MSU Crystals May Not Be Seen -Maintenance (preventive) dose 0.6 mg qd or bid.
-Needle in crystal-less sac. -0.3 mg 2-3 times per week in dialysis patients
-Ultramicroscopic size (need EM). (preventive).
-Spherules.
-Settled out. Systemic Steroids
-Lack of time to search. -Polyarticular attacks or fever.
-Lack of experience. -Longstanding attacks (>3-5 days).
-Need divided doses.
-Taper over 7-10 days.
-Start prophylactic agent (colchicine) as soon as possible.
Radiographic Hallmarks of Gout
-Overhanging edges Anakinra (Off-Label)
-Punched out lesions with sclerotic borders. -Effective for acute attack in studies.
-Preservation of joint space (till late) -Best in pts who cannot take steroids or colchcine.
-Degenerative changes -Expensive but 1 week of treatment may be affordable.
-Not for preventive use.
-Other interleukin-1 inhibitors currently in trials
(rilonacept & canakinumab)

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Adjunctive Measures Hypouricemic Therapy
-Rest -Don’t start hypouricemic agent during acute attack.
-Ice -Use probenecid first; it’s safer.
-Elevation -Don’t use probenecid if:
-Analgesics -overproducer
-Anti-motility agents (if using colchicine or indomethacin) -creat clearance < 35-50 ml/min.
-Continue hypouricemic agent if patient has been taking -history of kidney stones.
it.

Reasons for Hypouricemic Treatment Failure

Phase 2 - Preventive Therapy -Need lower uric acid levels than “normal.”
-Colchicine or NSAID. -Non-compliance.
-Always use when beginning a hypouricemic drug. -Renal insufficiency.
-Continue several weeks to years (depending on tophi, -Rapid dissolution of tophi.
serum uric acid). -Rapid elimination of oxypurinol (may occur with
-Always use before surgery in previously gouty patient. combined allopurinol and probenecid).

Phase 3 - Hypouricemic Therapy Asymptomatic Hyperuricemia

-Not every patient needs it. -Don’t treat it (this advice may change in future)
-May not need it in: -Exception: Patients getting chemotherapy for leukemia,
-Very elderly lymphoma.
-Non-compliant
-Infrequent attacks and no tophi
-May exacerbate attacks early on
Major Toxicities of Allopurinol
-Increased gout attacks early on (use prophylaxis)
-Rash (may be severe)
-Stevens-Johnson syndrome
-Vasculitis
Goals of Hypouricemic Treatment -Hepatitis
-Aim for serum uric acid under 6, preferably near 5 for -Renal failure (interstitial nephritis)
some chronic gouty patients. -Bone marrow suppression
-But remember:
-allopurinol toxicity more likely w higher dose.
-More likely with renal insufficiency.
Allopurinol Hypersensitivity Syndrome
-Fever
-Rash
Hypouricemic Agents -Renal Failure
-Allopurinol -Hepatic injury
-Febuxostat -Leukocytosis
-Probenecid -Eosinophilia (the tipoff!)
-Pegloticase
-Losartan (off-label) -May be fatal. Hard to treat.
-High-dose salicylates (off-label) - Serious reactions to allopurinol reported in 1 of 260
-Vitamin C (off-label) patients. Arthritis Rheum 29:82, 1986

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Treatment of Stones in Gouty Patients Resistant Hyperuricemia?
-Try febuxostat 40 mg BID instead of 80 mg qd (off-label
-Allopurinol use).
-calcium and uric acid stones -Short half-life supports this dosing.
-Currently in clinical trials
-Potassium citrate
-calcium and uric acid stones
-direct inhibitor of nucleation
-Fluids Losartan & Vit C (Off-Label)
-Lowers uric acid 0.3 – 1.3 mg/dl (dose range 25 – 200
mg/d).
-Uricosuric mechanism.
Febuxostat
-Useful when 24 hour uric acid is < 800 mg/d.
-Non-xanthine inhibitor of XO and XD.
-Maintain good hydration.
-Better tolerated than allopurinol.
-Effect is not seen with other ARBs.
-Lower uric acid levels than allopurinol (53% vs. 21% met
-Also consider fenofibrate (quite good actually) and
target of 6.0 mg/dl).
atorvastatin (both off-label).
-Better dissolution of tophi.
-Don’t forget vitamin C (500 mg BID)
Adverse Reactions
-Nausea
-Gout flare (must be on prophylaxis!)
-Elevated ALT, AST (3% > 3xULN)
-Elevated CRP Gout vs. Pseudogout
-Rash Gout
-Elevated CK -hallux, ankle, knee, hand
-younger, male
Febuxostat: Best Use
-Allopurinol failures Pseudogout
-Renal insufficiency -knee, wrist, ankle
-Tophaceous gout -older, female

-Almost any joint can be affected by either disease!

Allopurinol & Febuxostat Drug Interactions

-Life threatening interaction with azathioprine, 6-
mercaptopurine.
-Reduce dose of purine analogue by approximately 2/3.
Screening Films to Get in Pseudogout Patient
-Theophylline
-Knees
-Other interactions also
-Pelvis
-Hands

Pegloticase
-For refractory chronic gout
CPPD Deposition
-Dissolves tophi in weeks to months
-Wrist: triangular ligament
-Problems:
-Pelvis: symphysis pubis
-Anaphylaxis
-Knee: menisci
-Antibody formation
-Also: annulus fibrosis, articular capsules, bursae,
-Not in G6PD defic.
ligaments, tendons

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Clinical Associations with Psuedogout The Basic (Non-Acidic) Calcium Phosphates
-Aging -Hydroxyapatite
-Previous joint surgery -Calcium carbonate
-Previous joint trauma -Octacalcium phosphate
-Familial types -Tricalcium phosphate (whitlockite)
-Gout -Hydroxyapatite is non-birefringent.
-Amyloidosis
-Hyperpara
-Hemochromatosis
Syndromes Associated with Hydroxyapatite
-Hypomagnesemia
-Acute monoarthritis (pseudopseudogout)
-Familial hypocalciuric hypercalcemia
-Acute calcific tendinitis, bursitis
-Hypophosphatasia
-Scleroderma, dermatomyositis
-Wilson’s disease
-Heterotopic calcification
-Ochronosis
-Milwaukee shoulder
-Crowned Dens Synd.

Pseudo-DJD Pattern of CPPD Acute Apatite Monoarthritis

-50% of CPPD patients. (Pseudopseudogout)
-Wrists, MCPs, elbows, shoulders, knees. Note difference -Is usually a peri-arthritis.
from usual DJD pattern. -Intense inflammation (looks septic)
-Heberden’s or Bouchard’s frequently found. -Synovial fluid often non-inflammatory.
-May be acute or chronic. -Often causes podagra (especially in younger women).
-Look for the telltale calcifications on radiographs.

Treatment of Acute Psuedogout

-Aspiration (more important than in gout!) Summary
-Rest -Always give prophylaxis (colchicine or NSAID) before
-Intra-articular steroids reducing uric acid.
-NSAIDs -Longer courses of prednisone in divided doses for severe
-Systemic steroids gout.
-Colchicine? -Consider anakinra for acute treatment in some cases.
-IL-1 Inhibitors? -Febuxostat is more effective than allopurinol in renal
insufficiency.
-Pseudopseudogout mimicks gout in young persons.
-CPPDD is associated with destructive osteoarthritis;
Pseudogout Prevention consider methotrexate.
-Colchicine
-NSAID
-Magnesium?
-There’s no allopurinol for pseudogout (unfortunately).

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 -CXR : WNL
The Vasculitis Syndromes
-Blood C/S – sterile (3 samples)
22yrs lady, resident of Dharan-16, student of class XII
Echocardiography :
Presented in ER with c/o:
-Mod MR ( ? Bicuspid valve), pressure gradient across
1. Dizziness followed by brief loss of consciousness for 5 valve 13.4 mm Hg
yr, increased in frequency for last 1 month

-H/o pain upper limbs on washing clothes.

Provisional diagnosis
-No h/o palpitation, chest pain, shortness of breath
-Takayasu’s Arteritis
-No h/o abnormal movements of the body, tongue bite,
Stool or urine incontinence, weakness of any part of
body, facial deviation
Treatment :
-Tab aspirin 150mg PO OD PC
-Diagnosed as RHD- mild AR at Simala 1 yr back -Referred to Higher Centre
No h/o HTN, DM
-Pt on Inj. Benzathine penicillin 1.2 mU IM 3wkly
-Non smoker, non- alcoholic

O/E:
The Vasculitis Syndromes
-Pallor, icterus, cyanosis, clubbing, dehydration,
lymphnodes, oedema – Absent Definition
BP- 120/70 mmHg (Rt. Post. Tibial) -Vasculitis: clinicopathologic process characterized by
PR: 80bpm Rt. Arteria dorsalis pedis, regular, normal inflammation of and damage to blood vessels
volume and character. 1. Primary
B/L radial , brachial pulse – Absent 2. Secondary
B/L carotids feeble
B/L femoral, popliteal, post. Tibial & arteria dorsalis
pedial – palpable
RR: 16/min Primary vasculitis syndromes
T – 98 F -Granulomatosis with polyangiitis
SPO2 : 95 % in room air -Churg-strauss syndrome
Carotid Bruit (Rt.) + -Polyarteritis nodosa
-Microscopic polyangiitis
Chest : B/L equal air entry, NVBS, no added sounds -Giant cell arteritis
CVS : S1, S2 NAD, no gallops, no murmurs -Takayasu’s arteritis
P/A : soft, non tender, no organomegaly, BS +, No renal -Henoch-Schonlein purpure
bruit -Idiopathic cutaneous vasculitis
CNS : oriented to TPP, no focal neurological deficit, no -Essential mixed cryoglobulinemia
cerebellar signs -Behcet’s syndrome
-Isolated vasculitis of central nervous system
Investigation -Cogan’s syndrome
-TC : 11,600 N67 L33 Hb : 12.6% Platelets : 500000/mm3 -Kawasaki disease
st
-ESR 40 mm in 1 hr
-RBS : 126mg%
-Urea : 22 mg% Cr. : 0.7mg%
-Na : 146mmol/L K: 4.2 mmol/L
-ECG : WNL

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Secondary vasculitis syndromes cryoglobulinemia
-Drug induced vasculitis -Hepatitis B – associated polyarteritis nodosa
-Serum sickness
-Vasculitis associated with other primary diseases b. Production of antineutrophilic cytoplasmic antibodies
- infection, malignancy, rheumatic disease -Wegener’s granulomatosis
-Churg-Strauss syndrome
-Microscopic polyangiitis

c. Pathogenic T lymphocyte response and granuloma

formation
Classification as per vessel size -Giant cell arteritis
-Takayasu’s arteritis
Large Vessel
-Granulomatosis with polyangiitis
-Giant Cell Arteritis
-Churg-Strauss syndrome
-Takayasu’s arteritis

Medium Vessel
-Polyarteritis Nodosa Approach to a patient with suspected diagnosis of
-Kawasaki disease vasculitis
Small Vessel
-Wegener’s Granulomatosis
-Microscopic Polyangiitis
-Churg-Strauss Syndrome
-Henoch-Schönlein Purpura
-Isolated cutaneous Vasculitis

Potential mechanisms of Vessel damage in Vasculitis

Syndromes

a. Pathogenic immune complex formation and/or

deposition
-Henoch-schonlein purpure
-Vasculitis associated with collagen vascular diseases
-Serum sickness and cutaneous vasculitis syndromes
-Hepatitis C – associated essential mixed

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TAKAYASU’S ARTERITIS Investigations
-Raised ESR
-Dr. Mikito Takayasu : 1905 -Hypoalbuminemia, increased levels of fibrinogen,
-Pulseless disease ; Aortic arch syndrome alpha2-globulin, gamma-globulin
-Inflammatory and stenotic disease of medium and large -Urinalysis – may be consistent with nephrotic syndrome
sized arteries
Imaging studies
Incidence and prevalence: -Angiography
-1.2-2.6 cases per million complete aortic arteriography
-Prevalent in adolescent girls and young women magnetic resonance arteriography
-More common in Asians

Isikawa criteria (1986)

Clinical Manifestations of Arterial Involvement in -age < 40yrs at diagnosis or onset of characteristic sign
Takayasu’s Arteritis and symptoms of 1 month duration in pt’s history
-Involvement of Lt. & Rt. Mid subclavian artery, with the
most severe stenosis or occlusion present in the mid-
portion from a pt. 1 cm proximal to the Lt. and Rt.
Respectively
-Annuloaortic ectasia or aortic regurgitation as shown by
angiography or echocardiography

American College of Rheumatology (ACR 1990)

-Angiographic criteria must show narrowing or occlusion
of the entire aorta, its primary branches, or large arteries
in the proximal upper and lower extremities
-Changes can include stenosis, occlusion or aneurysm
-Changes are not due to arteriosclerosis, fibromuscular
dysplasia or similar causes
Types -Changes are focal or segmental
Type I ( Shimizu-Sano)- aortic arch and brachiocephalic
vessels
Type II (Kimoto) – thoracoabdominal aorta and renal
Mangement
arteries
Type III (Inada) – features of Type I & II Medical therapy:
1. Corticosteroids
Progression through 3 stages:
prednisolone 1mg/kg/day
1. First stage (Prevasculitic) – constitutional symptoms
2. Cyclophosphamide 2mg/kg/day PO, 1gm/m2/month
2. Second stage (Vascular inflammatory stage)
3. Methotrexate 0.3 /kg/wk increased upto 25mg
3. Third stage (burned out stage) - fibrosis
4. Antiplatelet agents : to prevent CVA
Causes : Aspirin, Clopidogrel
-Aetiology unknown.
Procedures:
-Several etiologic factors proposed :
1. Bypass surgery
spirochetes, *mycobacterium tuberculosis, streptococcal
2. Aneurysm clipping and revascularization
organisms, circulating antibodies due to an autoimmune
3. Angioplasty and stenting for recurrent stenosis
process

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Granulomatosis with polyangiitis Renal disease (77%):
-Mild glomerulitis with proteinuria, hematuria to rapidly
-Granulomatous vasculitis of the upper & lower progressive renal failure if untreated
respiratory tract together with glomerulonephritis
-Disseminated vasculitis involving both small arteries and Eye involvement (52%)
vein may occur -Mild conjunctivitis to dacrocystitis
-Episcleritis
Incidence: -Scleritis
-Male to female ratio 1:1 -Granulomatous sclerouveitis
-Mean age of onset – 40yrs -Ciliary vessel vasculitis
-Rare before adolescence -Retro orbital mass leading to proptosis

Skin lesions (46%)

-Papules, vesicles, palpable purpura, ulcers or
Histopathologic hallmark
subcutaneous nodules
-Necrotizing vasculitis of small arteries and veins
together with granuloma formation Cardiac involvement (8%)
-Lungs: B/l multiple, nodular cavitary infiltrates -Pericarditis, coronary vasculitis , rarely cardiomyopathy
-Upper airways: sinuses and nasopharynx –
inflammation, necrosis and granuloma formation with or Nervous system (23%)
without vasculitis -Cranial neuritis, mononeuritis multiplex or cerebral
-Kidney : focal or segmental glomerulonephritis vasculitis and/or granuloma
-May evolve into a rapidly progressive crescentric
glomerulonephritis
Lab findings
-Raised ESR
Clinical findings -Mild anaemia
-leucocytosis
Upper respiratory tract findings: -Thrombocytosis
-Paranasal sinus pain -Hypergammaglobulinaemia
-Purulent or bloody nasal discharge -cANCA +ve (90%)
-Nasal mucosal ulceration
-Nasal septal perforation – saddle nose Tissue biopsy:
-Serous otitis media -Necrotizing granulomatous vasculitis
-Subglottic tracheal stenosis

Treatment

1. Corticosteroids
- Prednisolone 1mg/kg
- With cyclophosphamide induction for severe disease
- 2mg/kg/day PO

After remission replaced with

Lower respiratory tract findings : (85-90%) -Methotrexate 0.3mg/kg/wk
-Cough, hemoptysis, dyspnoea, chest discomfort -Azathioprine 2mg/kg/day
-Mycophenolate mofetil 1gm BD

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CHURG-STRAUSS SYNDROME Treatment
-Described by Churg and Strauss in 1951 -Glucocorticoid
-Allergic angiitis and granulomatosis -Fulminant multisystem disease: combination of
glucocorticoid and cyclophosphamide
Characterised by : -Untreated 5yr survival 25 % and with treatment 78
-Asthma, peripheral and tissue eosinophilia, months survival 72%
extravascular granuloma formation
-Vasculitis of multiple organ systems

Incidence & prevalence : POLYARTERITIS NODOSA

-1-3 per million -Described in 1866 by Kussmaul and Maier
-Occurs at any age except infants; mean age of onset – -Multisystem, necrotizing vasculitis of small and medium
48 yrs sized muscular arteries
-Female to male ratio 1.2:1 -Involvement of renal and visceral arteries characteristic
-Segmental and tend to involve bifurcations and
branching of arteries
Aneurysmal dilatations upto 1 cm in size along the
Pathology and pathogenesis
involved artery
-Necrotising vasculitis involving small and medium sized -Arteritis without glomerulonephritis in kidneys
muscular arteries, capillaries, veins and venules -Hepatitis B antigenemia in 10-30% of pts.
-Granulomatous reaction in tissues and within the walls
of the vessels
Clinical manifestations

Organ system Clinical features

Clinical features
- non-specific manifestations Renal Renal failure, HTN
-Severe asthmatic attacks and presence of pulmonary Musculoskeletal Arthritis, arthralgia, myalgia
infiltrates Peripheral nerv system peripheral neuropathy,
-Mononeuritis multiplex (72%) mononeuritis multiplex
-Allergic rhinitis and sinusitis (61%) Gastrointestinal tract abdominal pain, nausea,
-Skin lesions (51%)- purpura, cutaneous and vomiting, bleeding, bowel
subcutaneous nodules infarction and perforation,
-Heart disease(14%) cholecystitis, hepatic
-Renal disease less common infarction, pancreatic infarction
Skin rash, purpura, nodules,
cutaneous infarcts, raynaud’s
Lab findings phenomenon
-Eosinophilia >1000 cells/mcl in 80% of pts. Cardiac CHF, myocardial infarction,
-Elevated ESR, fibrinogen, alpha2-globulins -81% pericarditis
-P-ANCA +ve -48% Genitourinary testicular or ovarian pain
CNS CVA, seizures

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Lab findings Clinical features :
-HBsAg +ve in 30% -When temporal artery involved, headache, tender,
-Angiographic findings of aneurysm of small and medium thickened or nodular artery
sized arteries in renal, hepatic and visceral vasculature -Scalp pain and claudication of the jaw and tongue may
-Biopsy from symptomatic organs- skin, testes, occur
nerves/muscles -Serious visual impairments- ischaemic optic neuropathy
-Claudication of extremities, stroke, myocardial infaction
-Aortic aneurysm and dissection
Treatment
-Glucocorticoids
-Severe cases : cyclophosphamide Lab findings:
-Treatment of hepatitis B -Elevated ESR
-Antihypertensive therapy -Anaemia
-Raised alkaline phosphatase
-Increased level of Ig G and complement
-Diagnosis confirmed by biopsy of temporal artery
MICROSCOPIC POLYANGIITIS
-Microscopic polyarteritis – Davson in 1948
-1992- Chapel Hill Consensus Conference on the
Nomenclature of Systemic Vasculitis adopted term Treatment
microscopic polyangiitis 1. Glucocorticoid therapy
-Mean onset 57 yrs, male slightly more frequently prednisolone 40-60 mg/day for 1 month and tapered
affected than female 2. Aspirin for cranial ischemic complications
-Glomerulonephritis and pulmonary capillaritis common- -Methotrexate as glucocorticoid sparing agent
renal failure and hemoptysis controversial
-Mononeuritis multiplex and gastrointestinal and
cutaneous vasculitis
-p- ANCA +ve (75%)
KAWASAKI DISEASE
-Mucocutaneous lymph node syndrome
-Acute febrile multisystem disease of children
-80% cases below 5yrs of age, peak incidence <=2 yrs of
age
GIANT CELL ATRERITIS -Non suppurative cervical lymph adenitis
-Cranial arteritis or temporal arteritis -Congested conjunctiva, erythema of oral cavity, lips and
-Inflammation of the medium and large sized arteritis palms
-Involves one or more branches of carotid artery -Desquamation of skin of finger tips
particularly temporal artery -25% cases associated with coronary artery anuerysm
-Closely associated with polymyalgia rheumatic
-Almost exclusively in individuals >50yrs of age Treatment
-More common in women than in men -High dose IV Gamma globulin
-6.9-32.8 per 100000 population 2g/kg as a single infusion over 10 h
-Association with HLA –DR4 -Aspirin 100mg/kg/day for 14 days followed by 3-5mg/kg
-Panarteritis with inflammatory mononuclear cells for several weeks
infiltrate within the vessel wall with frequent giant cell
formation

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Drug induced vasculitis Pulmonary Tuberculosis GPA
-Palpable purpura –generalized , lower extremities or
dependent areas Apical Infiltrates Mid Zone Infiltrates
-Fever, malaise or polyarthralgia
Upper Airway Disease -Rare Upper Airway diseases -
-Allopurinol, gold, thiazides, phenytoin, sulfonamides and
Common(40-80%)
penicillin
-Associated with antimyeloperoxidase ANCA – Pleural Effusion -Common Pleural Effusion -
hydralazine, propylthiouracil Uncommon
-Glucocorticoid and cyclophosphamide
Renal Involvement -Rare Renal Involvement -
Common
When to Suspect Vasculitis? Pericarditis & Pericardial Coronary arteritis & CHF
Prolonged Unexplained Systemic Illness
ffusion
Pyrexia of Unknown Origin(PUO) Common
Palpable Purpura Common
Pulmonary Infiltrates
Ocular Diseases –Rare Ocular Disease -Common

Viral Infections can Mimic Many Vasculitic Syndromes

-Hepatitis B—Polyarteritis nodosa
-HIV—“Seronegative” Rheumatic Syndromes

GPA & Tuberculosis have Several Common Clinico

Radiological Features

Clinical features Pulmonary GPA

Tuberculosis

Constitutional ++ ++
Symptoms

Cough with ++ ++
Sputum

Hemoptysis + +

Sub Acute + +
Clinical course

Rapidly Fatal - ++

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 How it Happens
Heat Stroke, Cold Injuries And
Drowning Stage One
-Activity and outside heat sources raise the body’s core
temperature.
-Heated blood pumps to the outer layers of body, where
Pathophysiology it radiates and conducts heat to the environment, if
cooler.
Thermoregulation
-If this fails to cool you, then you sweat through pores
-Body Temperature
on the skin surface, which evaporates and releases heat.
-Core temperature
-Peripheral temperature Stage Two
-The Hypothalamus -During heavy work, a body can lose 1-2 liters of water
-Heat Dissipation per hour.
-Sweating, vasodilation -After 2-3 hours of fluid loss, a person is likely to:
-Heat Conservation -Become uncomfortable
-Shivering, vasoconstriction -Lose endurance
-Feel hot
-Sense thirst
It’s a Fine Balance
Stage Three
-The longer a body sweats, the less blood there is to
carry excess heat to skin or oxygen and nutrients to
muscles.
-After 3 hours, a dehydrated worker may experience:
-Headaches
-Muscle fatigue
-Loss of strength
-Loss of accuracy and dexterity
-Heat cramps
-Reduced alertness
-Nausea

Causes of Heat Illness

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Heat Cramps Heatstroke can be

Cause: Temporary fluid electrolyte imbalance. Low salt Non-exertional (classical)

with heavy physical exertion -Slow evolution, onset insidious
-usually affects elderly and debilitated patients with
Symptoms: Painful muscle spasms in the arms, legs, and chronic underlying disease.
abdomen - Result of impaired thermoregulation combined with
high ambient temperatures. Often due to impaired
Consequences: With heavy sweating and thirst may
sweating
signal heat exhaustion
-Increases exogenous heat gain with decreased heat
Rx: Rest in cool environment; replace water and salts dispersal
with sports drink. -Increased risk with
-CV disease
-Older age
-Cardiovascular/anticholinergic drugs

Exertional
Heat Stroke
-Due to vigorous activity
Definition -typically seen in healthy young adults who overexert
-“Characterized by elevated core body temperature themselves in high ambient temperatures or in a hot
above 40 degrees Celsius and central nervous system environment to which they are not acclimatized
dysfunction that results in delirium, convulsions, or -Sx same as for non-exertional
coma.”
-“Form of hyperthermia associated with a systemic
inflammatory response leading to a syndrome of Factors precipitating heat stroke
multiorgan dysfunction in which encephalopathy -High temperature
predominates.” -Elderly patients
-Dehydration
-Decreased sweating
Heat stroke Triad -Obesity
0 0
-Temp > 40.5 C (104.9 ) -Alcoholism
-CNS dysfunction
-Anhidrosis
Clinical features
Any neurological disturbance can occur with heat stroke.
-Onset: acute or insidious
-Diminished sweating and intense thirst
-Impairment of sensorium
Heat Stroke vs. Heat Exhaustion -Involuntary movements with convulsions
-Rapid bounding pulse
HEAT STROKE -Skin- hot, flushed and dry
1. Dry, hot skin -Petechiae, jaundice
2.Very high body temperature -Arrhythmias- AF
3. Confusion -Organ Failure.
HEAT EXHAUSTION
1. Moist clammy skin
2. Normal or subnormal body temperature

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Lab findings Supportive
-Haemoconcentration, leukocytosis -IV volume replacement
-High chloride, low potassium,calcium,phosphate -Inotrope --- dobutmine(if required)
-LFT- abnormal -Treatment of hyperkalaemia
-Features of coagulopathy/DIC - Do not treat hypocalcaemia as calcium may exacerbate
-ECG: non-specific rhabdomyolysis.
-Small dose of mannitol may benefit patients with
rhabdomyolysis
First Aid for Heatstroke or Sunstroke
-HEATSROKE IS LIFE THREATENING!
-Remove victim to cooler location, out of the sun Preventing heat-related illness
-Loosen or remove clothing and immerse victim in very
cool water if possible -Dress for the heat — Wear lightweight, light-coloured
-If immersion isn't possible, cool victim with water, or clothing. Light colours will reflect away some of the sun’s
wrap in wet sheets and fan for quick evaporation energy. It is also a good idea to wear hats or to use an
-Use cold compresses-especially to the head & neck area, umbrella.
also to armpits and groin -Drink water-
- Avoid alcohol and caffeine, which dehydrate the body.
-Avoid foods that are high in protein, which increase
metabolic heat.
-Stay indoors when possible.
-Take regular breaks when engaged in physical activity on
warm days.
-Take time out to find a cool place.

Once in the hospital, an examination is done, and blood

tests are carried out to assess the level of salts in the Hypothermia
blood. -Core temp—less than 35 degree celcius.
-Treatment of heat stroke is usually carried out in a
Types-
critical care unit.
-Primary-
-The body temperature is lowered by sponging the body
-Secondary
with tepid water or loosely wrapping the person in a wet
sheet and placing him or her near a fan.
-Intravenous fluids are given.
-when temperature approaches 39° active cooling should Types of Cold Injuries
be terminated as the body temperature will continue to -Hypothermia
fall 1-2° C -Frostbite
- chlorpromazine 10-50 mg IV 6hrly may be useful in -Chilblains
preventing shivering -Immersion/Trench Foot

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Hypothermia ECG
-Due to universal lowering of body temp as a result of -Pathognomonic J waves (Osborn waves)
acute exposure to severe cold -Eventual onset of bradycardia
-Systemic Ds.- pitutary, thyroid, MI -Ventricular fibrillation probable below 86º F
-Immersion hypothermia- cold water
-Mistaken for dead.

Treatment
-Insulation of body-blanket or sleeping bag
0
-Oxygen at 42-44 c with 100% humidity
-Expand blood volume

Rewarming:
-External means: blankets, forced hot air, warm bath
-Internal means: humidifying inspired air/oxygen
mixtures, warm fluid lavage of body cavities,
hemodialysis and cardiopulmonary bypass

Clinical features Frostbite

0
-At 35 C- shivering
0 a. Superficial Frostbite
-Below 35 C - voilent behaviours, clouding of mental
-Freezing of epidermal tissue
status, slurring of speech, fainting
0 -Redness followed by blanching and diminished
-Below 30 C- cessation of all cerebral activities, pulse and
sensation
respiration becomes slow
0
-Below 27 C- unconscious b. Deep Frostbite
0
-Below 25 C- comatosed, pulmonary edema, generalised -Freezing of epidermal and subcutaneous layers
rigidity -White, frozen appearance

Investigations
-Hemoconcentration, acidosis, azotemia
-TFT- depressed
-J wave (Osborn), prolonged PR, QT, AF, VF

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-Toes and feet Chilblain:
-Tissue freezing-ice crystals-damage cells -vasospastic reaction to cold
-Separation of dermal-epidermal interface -Toes become red and intense itching
-Exudation, edema, hemorrhage -Later cyanotic and intense pain
-Blister, blebs- local gangrene -May precede trench foot

Treatment
-Do not thaw the affected area if there is the possibility
of refreezing.
-Do not massage the affected area.
Drowning
-Administer analgesia prior to thawing.
-Drowning – death within 24 hours
-Transport; rewarm by immersion only if transport is
-Near-drowning – survival past 24 hours
lengthy or delayed.
-Cover the thawed part with a loose, sterile
dressing.
-Elevate and immobilize the thawed part. Three Peaks
-Do not puncture or drain blisters. -Toddlers
-Do not rewarm feet if walking will be required. -Adolescents
-Elderly

0 0
-Rewarm injured limbs- warm water bath 37 C-43 C
-Bed-rest, limb elevation, T.T inj. Severity factors:
-Heparin may decrease tissue loss - Water Tonicity
-Others: Aspirin, dipyridamole - Time submersion
- water Temperature
- symptoms associated injuries .
- Undetected primary cardiac arrhythmia( long QT)

Trench Foot
Drowning begin with:
Occurs above freezing.
1. Panic, breath holding, air hunger
-Typically occurs from standing in cold water.
2. reflex aspiration.
Symptoms are similar to frostbite. 3. laryngospasm that leads to hypoxemia
-Pain may be present, and blisters may form 4. hyperventilation followed by voluntary apnea .
with spontaneous rewarming.

Treatment:
Pathophysiology
-Warm, dry, and aerate the feet.
Prevention is the best treatment. Asphyxia may occur with:
1. pulmonary aspiration (wet drowning).

2. laryngospasm (10-20%)
until cardic arrest
)dry drowning)

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Anoxic-ischemic injury Near-Drowning and Drowning
-All organs may injured from hypoxia and ischemia .
Pathophysiology of Drowning and Near-Drowning
-CNS injury
-(ICP ,cerebral edema) -Dry vs. Wet Drowning
-The most frequent cause of mortality and
-Fresh-Water vs. Saltwater Drowning
long- term morbidity
-Fresh water causes the alveoli to collapse from
a lack of surfactant.
-Salt water causes pulmonary edema and
-Pulmonary: eventual hypoxemia due to its hypertonic
- wash out surfactant nature.
- Pulmonary edema, ARDS

-Cardiovascular:
-Arrhythmia( hypothermia ,hypoxemia) Dry-drowning
-10-20% of submersions
-Acid-base -Laryngospasm
-Hypoxia
-Electrolytes -Loss of consciousness

-Renal Wet-drowning
-ATN (hypoxemia,shock, hemoglobinuria) -Aspiration of water
-Dilution of surfactant
-Gasterointestinal
-Diminished gas transfer
-hepatic trasaminases and serum pancratic
-Atelectasis
enzymes are often acutely elevated
-Ventilation perfusion mismatch
-Pulmonary aspiration occurs in the great majority of -Near-Drowning
submersion .

-Pneumonia may result from :

Treatment for Near-Drowning
- gastric contents
-Remove the patient from the water.
- water salinity
-Initiate ventilation while the patient is still in the water.
- pathogenic organisms
-Suspect head and neck injury if the patient experienced
- toxic chemical
a fall or was diving.
-Place the victim on a long spine board and use c-spine
precautions throughout care.
The great majority of submersion do not aspirate large -Protect the patient from heat loss.
volumes of fluid to result in significant electrolyte -Evaluate ABCs.
disturbances. -Begin CPR and defibrillation if indicated.
-Manage the airway using proper suctioning and airway
-Sea water
adjuncts.
-Fresh water -Administer oxygen at 100% concentration.
-Use respiratory rewarming, if available.
-Establish IV of lactated Ringer’s or normal saline at 75
mL/hr.
-Follow ACLS protocols if the patient is normothermic.
-Treat hypothermic patients according to hypothermia
guidelines.

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Prevention

-Infants
-Parental vigilance
-Toddler
-Pool fencing
-Adolescent/Young Adult
-Control Alcohol/Drug Use
-Swimming lessons
-Elderly
-Same as infant/toddler

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 Physics
Electrical and Radiation Injury
-Electrical energy -> deposited as heat
Definitions -Heat causes the most tissue damage
-Electric shock – response
Joule’s Law
-Electrocution – death 2
Energy = I x R x time
-Electrical injury 2
Energy = (V x time) / R
– tissue damage
-Therefore the heating of tissues increases according to
-Electrical burn
the square of the applied voltage, and is directly
– cutaneous injury
proportional to the time the voltage is applied.

Principle Of Electricity
Electricity
-Electricity: flow of electrons (negatively charged outer
Power Line: 7620V
particles of an atom) through a conductor
Lines outside house: 220 / 240V
-When the electrons flow away from this object through Subway: 660V
a conductor they create an electric current: amperes
High Voltage Injury
-Voltage: force that causes electrons to flow: volts
->1000 V
-Anything that impedes the flow of electrons through a
-Severe skin burns
conductor creates resistance: ohms
Low Voltage Injury
Physics
-Cutaneous burns often minimal with household
-Electric flow / current = Amp
voltage, unless several secs contact
-Electric potential difference = Volts
-Electrical burns absent in 40% of low voltage
-Resistance = Ohms
deaths
-Conductors: high fluid, electrolyte content – nerves and
-110V can cause V fib
blood vessels, sweaty skin, saliva, muscle
-Insulators: high resistance – bone, dry skin

Ohm’s Law Lightning is a form of DC

I (Current) = V (Voltage) / R (Resistance) -Occurs when electrical difference between a
-Current is directly proportional to potential difference, thundercloud and the ground overcomes the insulating
and inversely proportional to resistance. properties of the surrounding air
-Example = Grasp 120V source, with 1000Ohms -Current rises to a peak in about 2 µsec
resistance = 120mAmps -Lasts for only 1-2 sec

Electrical current exists in 2 forms Severity of injury depends on

-Intensity of electrical current (voltage of source and
1) AC: (Alternating Current): when electrons flow back
resistance of victim)
and forth through a conductor in a cyclic fashion
-Pathway through victim’s body
-It is used in household and offices and is standardized to
-Duration of the contact with the source
a frequency of 60 cycles/sec (60 Hz)

2) DC: (Direct Current): when electrons flow only in one

direction
-Used in certain medical equipment: defibrillators,
pacemakers, electrical scalpels

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Effect of Current

Effect Current Path Minimum

Current 60 Hz
AC mAmp

Tingling Through intact 0.5-2

sensation, skin
minimal
perception

Pain threshold Through intact 1-4

skin

Inability to let go: From hand, 6-22

tetany decreases through Electrical Injuries
resistance forearm into Patterns of Injury
trunk
Skin Resistivity
Least Nerves
Respiratory Through chest 18-30 Blood
arrest: fatal if Mucous membranes
prolonged Muscle
Intermediate Dry skin
V Fib Through chest 70-4000 Tendon
Fat
Ventrical asystole, Most Bone
Through chest >2000
if current stops
sinus rhythm may
resume

Pathway of the current through the body:

Electrical Injuries -Vertical pathway parallel to the axis of the body is the
most dangerous. It involves all the vital organs; central
Factors Determining Severity nervous system, heart, respiratory muscles, in pregnant
2
women the uterus and fetus
Skin Resistivity - Ohms/cm
-Horizontal pathway from hand to hand: the heart,
-Mucous membranes 100 respiratory muscles and spinal cord
-Vascular areas 300-10000
-volar arm, inner thigh -Pathway through the lower part of the body: local
-Wet skin damage
-Sweat 1200-1500
-Bathtub 2500
-Other skin 10000-40000
-Sole of foot 100000-200000
-Heavily calloused palm 1000000-2000000

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Cardiovascular Injuries Spinal cord
-1° cause of death from electrocution -Vertebral fractures – multilevel!
-Low-voltage -> v fib -Delayed injury
-High-voltage AC and DC -> transient asystole -ascending paralysis
st nd
-Also: ST, PACs, PVCs, a fib, 1 / 2 AV block -complete or incomplete cord
-Vigorous resuscitation!!! -transverse myelitis
-Victims are often young without CVD -MRI results not closely correlated to outcome
-Not possible to predict outcome based on
rhythm
-Vascular injury -> spasm -> delayed thrombosis or
Eye Injuries
aneurysm formation, compartment syndrome
-Cataract formation weeks to years later
-Retinal detachment, corneal burns, intraocular
hemorrhage, intraocular thrombosis
Orthopedic Injuries / MSK
-Fractures 2° to tetany, falls
-Shoulder dislocation (voltages >110V)
Ear Injuries
-Muscle +++heat -> periosteal burns, osteonecrosis
-Late complications of hemorrhage into TM, middle ear,
-Severe arterial spasm -> compartment syndrome
etc. -> mastoiditis, sinus thrombosis, meningitis, brain
-Muscle breakdown -> rhabdomyolysis -> myoglobinuria
abscess
and renal failure
-Hearing loss immediate or late
Cutaneous Wound
-Entry / exit wounds – painless, gray
GI injuries
Treatment
-Suspect in patients with burns of abdo wall, or trauma
-Cleansing, Td
-Lethal injuries – reported only at autopsy
-Silver sulfadiazine
-Gastric ulcers – Curling’s ulcers
-Mafenide
-Fluid resuscitation -> abdominal compartment
-Full-thickness burns – penetrates eschar
syndrome with restrictive surface burns
-<25% BSA only – inhibits carbonic anhydrase,
painful
-Observe for neurovascular compromise, compartment
syndrome DIC
-Splint extremities, early surgical debridement, vascular -May be due to thermal injury or tissue necrosis
reconstruction and skin graft -Low-grade DIC from hypoxia, vascular stasis,
rhabdomyolysis, release of procoagulants
-Tx: eliminate precipitating factor by early surgical
debridement
CNS and Peripheral Nerve Injuries
-FFP or cryo as needed
-50% have impairment (high-voltage)
-Transient LOC
-Agitation, confusion,
-Coma
-Seizures
-Quadriplegia, hemiplegia, paresthesias
-Aphasia, visual disturbances

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MANAGEMENT No Admission:
-Household voltage injury 100-220V in adult +
Accident Scene: -Neglibible risk for delayed arrhythmias +
-Asymptomatic, normal ECG and normal exam -> d/c
A. Downed Power Lines

B. Rescuer
-Electrocution possible, recommend 9m away (3m may Summary: Electrical Injuries
be enough)
a. Low-voltage <600V -> may be D/C if asymptomatic
C. Victims -Immediate cause of death: V Fib
-Victims in contact with source may be “active” -Children: oral burns – consider labial artery bleed
-Voltage >600V -> dry wood, rubber boots may conduct ? admission
electricity
b. High-voltage >1000V -> admit for observation and
cardiac monitoring
-Asystole, treat cardiac arrest vigorously
ED Treatment
-Deep tissue destruction with high fluid needs
A. Resuscitation -Myoglobinuria and renal failure is common
-ABCs as per trauma -Trauma: thrown
-ACLS -Immediate cause of death: Apnea
-Spinal immobilization
-Careful physical exam!

B. Investigations
-Labs: High-voltage, extensive burns, evidence of
systemic injury
-CBC,electrolytes, Cr, BUN, CK, serum / urine myoglobin Lightning Injuries
-Imaging as indicated, clear spines -Lightning is extremely high-voltage DC
-CV: Causes asystole in arrest
C. Fluid Resuscitation
-Neuro: Apnea from medulla injury,
-Fluid requirements.
-MSK: Explosive effect of shock wave
-Visible damage < internal damage!
st
-Cutaneous: Lichtenberg figures
-Initial fluid bolus: 20-40mL/kg/ 1 hr
-Tx: ABCs, treat sickest first (even ?dead!)
Considerations: -Get ocular and neuro assessment
-Fluid load to prevent rhabdomyolysis -Admission for observation
-Avoiding over-resuscitation in patients with restrictive
burns on abdomen -> prevent compartment syndrome

Admission:
-In contact >600V
-Symptoms (chest pain,palpitation , LOC, confusion,
weakness, dyspnea, abdo pain)
-Signs (weakness, burns with subcut damage, vascular
compromise)
-Ancillary changes (ECG, CK, myoglobinuria)
-Cardiac monitoring: If ECG abnormal

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Sources of Radiation Sources of Alpha particles include:
a. Natural -americium-241
-Many more sources of natural radiation -radon
-Insignificant risk associated with typical exposure -plutonium
b. Man made -radium
-Fewer sources of exposure -thorium
-BUT…potentially deadly if misused -Uranium

These radionuclides all release Beta particles:

-cesium-137
Radiation -cobalt-60
a. Ionising radiation : -iodine-129 &-131
-Alpha particles -strontium-90
-Beta particles -Tritium
-X-rays , gamma rays
-Neutrons Gamma Rays
b. Non-Ionising radiation: -Electromagnetic radiation similar to medical x-rays
-UV Rays, -Short wavelength and high frequency
-visible light, -Can travel up to a mile in open air
-laser,infrared and microwave -Can penetrate deep in the tissue
c. Man Made Radiation (11%) -Most hazardous from sources outside the body
- Medical (ionising) -All tissues and organs can be damaged by sources
-X-rays outside the body
-CT scans
- Nuclear medicine
- Radionucleotide scan
Radiation Exposure
- Radiation oncology
Two major types of radiation exposure
- radiotherapy
-Dissemination of radioactive material without nuclear
-Non-ionising- UV therapy for skin disease, laser therapy
detonation
Properties Of Ionising Radiation -Nuclear detonation

Radiation Range in Air Range In Protection Whole body exposure

Tissue -External contamination
-Internal contamination
Alpha Few No Paper -Ingested
particles centimetres penetration -Inhaled
-Absorption from skin or wound
Beta Few metres Few Aluminium
particles millimetres sheet Localized exposure

X- Kilometres Passes Lead

rays/gamma through
rays

Neutrons Kilometres Passes Concrete or

through thick
polythene

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Measurement of Radiation Dose SI uses sievert (Sv) instead of rem
-What needs to be known for medical treatment - 1 Sv = 100 rem
-Intensity of exposure
-Time or duration of exposure SI units must be used on labels to identify radioactive
materials during transport

Roentgen

Biological Effects

-Depend upon type of exposure (duration of exposure)

-Acute (limited time of exposure)
-Chronic (extended or repetitive exposure)
-The Roentgen is used to express the amount of gamma
radiation exposure -Level of exposure (intensity)
-Abbreviated with a capital “R” after the amount of -Certain biological factors
gamma radiation received -Damage DNA and other structures inside cells
-Independent of the time of exposure -Could result in cell death
-Incorrect repair, resulting in mutations that could cause
Rad (radiation absorbed dose)
cancer
-Relates different types of radiation (alpha, beta, gamma
and neutron) to the energy they impart
-Basic unit of absorbed dose of radiation
-One roentgen of gamma radiation exposure results in Acute Exposure
about one rad of absorbed dose -Significant dose of radiation over a short period of time
-Radiation sickness or death shortly after exposure
Rem (roentgen equivalent man) -Long-term effects (possibly cancer years later)
-Relates the dose of any radiation to the biological effect
of that dose Chronic Exposure
-For gamma rays and beta particles, 1 rad of exposure -Small dose of radiation continuously or over many years
results in 1 rem of dose -No immediate observable effects
-For alpha particles, 1 rad of exposure results in ~20 rem -May result in long-term effects
of dose

Radiation effects

A.Deterministic effects
Exposure Rate -Increasing severity as dose increases above a threshold.
-The rate at which an individual is exposed to radiation -Tissues with actively dividing cells particularly sensitive .
-Expressed in terms of roentgen or milliroentgen per - Lymphocyte depletion - most sensitive marker of bone
hour marrow injury (major cause of death)
-Gastrointestinal mucosal toxicity may cause earlier
death due to profound diarrhoea, vomiting, dehydration
and sepsis.
International System of Units (SI)
-Gonads - temporary or permanent sterility.
SI uses gray (Gy) instead of rad -Eye exposure can result in cataract.
- 1 Gy = 100 rad -lung and central nervous system- acute inflammatory
reactions,.

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- Skin - radiation burns. Prodromal phase (within 4 days)
-Thyroid gland is not inherently sensitive but its ability to -Nausea/vomiting
concentrate iodine makes it susceptible to damage after -Headache
exposure to relatively low doses of radioactive iodine -Fatigue
isotopes -Fever, diarrhea
-Anorexia
-Fluid shifts
-Electrolyte imbalance
B. Stochastic effects
-Increasing probability of occurring as dose increases but Latent Phase (days to weeks)
severity is independent of dose. -Temporary improvement
-Carcinogenesis represents a stochastic effect.
-Epidemiological studies of the appearance of malignancy Manifest Illness (weeks to months)
after radiation exposure, e.g. after Hiroshima and -Intense immunocompromise and symptoms specific to 4
Chernobyl, are used to calculate the predicted risk after major organ systems (heme, GI, skin, neurovascular)
exposure.
-With acute exposures, leukaemia may arise after an Recovery or Death
interval of about 2 years and solid tumours after an
interval of about 10 years. Thereafter the incidence rises
with time. Note: After lethal dose, victims may go through these
-An individual's risk depends on the dose received, the phases in a period of hours resulting in early death
time to accumulate the total dose and the interval
following exposure

Acute Radiation Sickness Treatment

-The treatment of ARS is focused on maintaining
Acute Radiation Sickness homeostasis, giving damaged organs the chance to
-Occurs when an individual is exposed to a large amount recover.
of radiation in a short period of time -Treatment for the hematopoietic system includes mainly
-Tissues with high replication rate like bone marrow and therapy for neutropenia and infection, transfusion and
mucosal surface of GIT are more sensitive than slowly blood products as needed, and hematopoietic growth
dividing tissue like bones and muscle factors.
-Partial or total parenteral nutrition, to bypass the
Clinical Manifestation:
damaged GI system.
Three major group of sign and symptions
-Treatment of blast and thermal injuries
-Hematopoietic
-Psychological support
-Gastrointestinal
-Neurovascular

Management
-Primary Triage
Four Stages of ARS
-Prodromal phase (within 4 days) Radionuclide Decontamination
-Latent Phase (days to 2.5weeks) -Body surface: Change Cloth, Washing
-Manifest Illness (weeks to months) -Lung lavage
-Recovery or Death -GI clearance:
-Stomach lavage
- emetics (such as apomorphine, 5 to 10 mg, or

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 ipecac, 1- to 2-g capsules or 15 mL in syrup); Long-Term Effects
-purgatives, laxatives,
-ion exchangers: Prussian blue, 1 g tid for a
minimum of 3 weeks, used to treat cesium 137
internal contamination.

Prevention or reversal of radionuclide interaction with

tissues can be done by blocking, diluting, mobilizing,
and chelating agents.

Blocking agents:
- prevent entrance of radioactive materials.
-example KI which blocks the uptake of radioactive iodine
(131I) by the thyroid.
-Other thyroid-blocking agents

Mobilizing agents
-may be effective for up to 2 weeks after exposure.
- parathyroid extract, glucocorticoids, ammonium
chloride, diuretics, expectorants, and inhalants.

Diluting agents
-water may be used as a diluting agent in the treatment
for tritium (3H) contamination.

Chelating agents
-to treat internal contamination with plutonium,
americium and curium, but it also chelates berkelium,
californium, or any material with an atomic number >92.
-diethylenetriaminepentaacetic acid (DTPA) either as Ca-
DTPA or Zn-DTPA
-ethylenediamine tetraacetic acid (EDTA)

Bone Marrow Transplantation

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 -Serum levels peak 30 to 60 mins
Poisioning
-Absorption often delayed in overdose peak level 2-8 hrs
-Clinical factors influencing Toxicity

Pathways of metabolism

a. Major Route Phase 2 reaction

-Conjugation with sulfate(25-36%) and glucuronide (47-
62%)
-form nontoxic metabolites in liver

b. Minor route Phase 1 reaction

-Oxidized by hepatic cytochrome P450 enzymes(5-8%)
-Forms n-acetyl-p-benzo quinone imine (NAPQI)

Acetaminophen poisioning
-Over the counter drug
-Most common pharmacologic agent in toxicologic
fatalaties
-Recomended maximum dose in adult 4g/d
-Can lead to fatal hepatic necrosis

Pharmacokinetics
-Available in both immediate-release and sustained-
release formulations
-maximum recommended daily dose: 80mg/kg in
children, 4 g in adults
-Toxicity is unlikely to result from a single dose of less
than 150 mg/kg in a child or 7.5 to 10 g for an adult
-Toxicity is likely to occur with single ingestions greater
than 250 mg/kg or those greater than 12 g over a 24-
hour period

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NAPQI Phase III fulminant hepatic failure
-toxic, highly reactive, electrophilic intermediate -Liver function abnormalities peak from 72 to 96 hours
-rapidly conjugated with hepatic glutathione, forming after ingestion
nontoxic cysteine and mercaptate compounds that are -systemic symptoms reappear in conjunction with
excreted in the urine jaundice, confusion (hepatic encephalopathy),
-toxic doses sulfation and glucuronidation pathways are coagulopathy hypoglycemia
saturated, metabolized to NAPQI -ALT - often between 2000 and 10,000 U/L
-NAPQI arylates and binds covalently to the cysteine -Acute renal failure develops in some critically ill patients
groups on hepatic macromolecules, forming NAPQI- -Death from multiorgan failure may occur
protein adducts
-irreversible and leads to oxidative injury and Phase IV Recovery Phase 4 days to 2 weeks
hepatocellular centrilobular necrosis -Pts who survive stage III enter a recovery phase by day
four
-Histologic recovery lags behind clinical recovery
- centrilobular region (zone III) is preferentially involved;
Factors influencing toxicity the area of greatest concentration of CYP2E1; site of
-Excessive intake of acetaminophen maximal production of NAPQI
-Excessive cytochrome P450 activity
-Decreased capacity for glucuronidation or sulfation
-Depletion of glutathione stores

-Fasting, malnutrition, Anorexia

-Elderly Diagnosis
-Chronic alcoholism, febrile illness, chronic disease -Dose of 140-150 mg/kg is potentially toxic limit that
-P450 enzyme inducers (INH, phenytoin, smoking) requires therapeutic intervention
-If total dose cannot be obtained obtain APAP levels at 4
hours or later after ingestion
-APAP concentration is plotted on RUMMACK- MATHEW
Clinical features NOMOGRAM

Phase I (Asymtomatic Phase)

-0.5-24 hours after ingestion
-Patients may be asymptomatic or may anorexia, nausea
or vomiting, and malaise
-Physical examination may reveal pallor, diaphoresis,
Fatigue

Phase II ( Hepatotoxic Phase) 24-72hrs

-clinical and laboratory evidence of hepatotoxicity
become evident
-stage I symptoms may resolve and patients appear to
improve clinically
-over half demonstrate aminotransferase elevation
within 24 hours
-right upper quadrant pain, with liver enlargement and
tenderness
-Elevations of prothrombin time (PT) and total bilirubin,
oliguria, and renal function abnormalities may become
evident

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Kings college Criteria Acetylcholine is present through the autonomic and
-Predict the probability of developing severe Liver central nervous system, functioning as a chemical
damage neurotransmittor at pre and post ganglionic
-PH< 7.3 parasympathetic synapses , preganglionic sympathetic
-PT> 100, Sr Cr > 3.3 mmol/l, Severe hepatic synapses, neuromuscular junctions and in CNS.
encephalopathy
-Elevated Phosphate > 3.7 mg/dl
-Lactate >3 mmol/L

Management basic principles

If early presentation :
-activated charcoal 1g/kg
-Check, CBC, RFT, LFT, ABG, PCM TDM at 4 hours post-
ingestion

If 8-24 hours & suspicious of large overdose :

-Start N acetyl cysteine, stop if level below therapeutic
line & INR/LFT normal

-Cholinesterase enzyme rapidly hydrolyses acetylcholine

into inactive fragments of choline and acetic acid.

-RBC (True cholinesterase)

Organophosphorus Poisioning -Serum (False cholinesterase)

-Used as insecticides worldwide for more than 50 years -Inhibition of cholinesterase enzyme leads to excessive
-Common cause of toxicology related deaths in accumlation of Ach at synapses. This causes initially
developing world overstimulation (cholinergic crisis) and subsequently
-Organophosphorus are also potent chemical terrorism paralysis in both the central and peripheral nervous
and warfare agents system.

Muscarinic receptors-
-post ganglionic parasympathetic nerve endings
Organophosphorus Compounds – binds irreversibly to
the cholinesterase enzyme Nicotonic receptors-
Carbamates - binds reversibly to cholinesterase enzyme - autonomic ganglion
- skeletol myoneural junctions
Organophosphorus - phorate
chlorpyrifos
malathion
-Organophosphorus and carbamates produce a similar
Carbamates - propoxur (Baygon) clinical spectrum of poisoining and have identical
pathophysiological mechanisms.
-Generally carbamates produce less toxic and shorter
courses.

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Exposure Laboratory Findings
-These compounds are absorbed efficiently by oral, -No specific lab tests in emergency
dermal, conjunctival and gastrointestinal routes. -Decreased cholinesterase activity in blood----- both true
-Poisoining occurs commonly as a result of agricultural cholinesterase and false cholinesterase activity is
use, accidental exposure, sucidal (most common) and decreased
rarely homicidal. -True Cholinesterase activity is more accurate

Clinical Features Pathophysiology

-potent acetyl cholinesterase inhibitors
Muscarinic effects- -Accumulation of Ach at nicotinic and muscarinic
-hypersecretion of the salivary , lacrimal, sweat glands – receptors resulting in excessive cholinergic stimulation
excessive salivation,lacrimation, frothing from mouth,
pulmonary edema, diaphoresis.
-Miosis
-Nausea, vomiting, diarrhea Treatment
-Bradycardia -Decontamination and Supportive therapy
-Blockade of Muscarinic activity with ATROPINE.
SLUDGE -Reversal of cholinesterase inhibition with OXIME.
-Salivation -Correction of Metabolic abnormalities
-Lacrimation -Prevention of infection.
-Urination -Management of complication
-Diarrhoea
-Gastrointestinal symptoms Decontamination and supportive theraqpy
-Emesis -Comatose or vomiting patients should be kept in left
Lateral position,
-Patent airway :placement of airway or with
endotracheal intubation especially if
Nicotonic Effects -Frequent suctioning is essential
-Muscle fasciculations -Oxygen therapy can be assessed by assessment of
-Muscle cramping / Muscle paralysis arterial oxygen saturation.
-Hypertension, Tachycardia, Mydriasis--------are rare,
occurs b/o the overstimulation of the nicotinic receptors -All clothing, hair accessories are to be removed washed
in the sympathetic ganglia may overwhelm with copious amount of water and soap.
parasympathetic stimulation. -Ocular decontamination with water/normal saline.
-The health care workers need protection through
personnel protecting equipments. Rubber Gloves and
gowns
CNS Effects
-Confusion, coma , convulsions

Gastric lavage
-should be considered in patients presenting within 1-2
Most common cause of death is respiratory failure----
hours ofingestion of poison.
depression of CNS respiratory centre, weakness of the
-Risks of gastric lavage include aspiration, hypoxia, and
respiratory muscles, and increased bronchial secretions.
laryngeal spasm.

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Activated charcoal
-reduce the poison load by adsorbing it.
-Its efficacy has not been conclusively proven in humans.
-single to multiple dose activated charcoal
-AVOID cathartics and induced emesis

Atropine
-Specific antidote for muscarinic effects, no effect on
nicotinic symptoms.
It reverses life threatening features that can result in
death :
-central respiratory depression
-bronchospasm, excessive bronchosecretion
-severe bradycardia, and hypotension.

Target end-points for Atropine therapy :

-Heart rate >80/ min.
-Dilated pupils.
-Dry axillae.
-Systolic blood pressure >80 mm Hg.
-Clear chest on auscultation with resolution of
bronchorrhea (absence ofwheeze and crepts).

Recommended dose is an initial iv bolus of 1.8-3mg with

subsequent doses doubled every 5 minutes if there is no
response or repeat same dose until atropinization is
achieved.

Maintenance dose: 20% of initial atropinizing dose per

hour for first 48 hours and gradually taper over 5 -10
days,

Pralidoxime (PAM)
-It reverses the cholinergic nicotonic effects that are
unaffected by atropine alone.
-It also reactivates the cholinesterase enzyme.

-1 gm of pralidoxime in 100 ml of normal saline over 15-

30 minutes, 8 hrly
-Continuous iv infusion of pralidoxime (0.5 gm/ hr)

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 Classification of Snakes
Snake bite
Snakes are generally classified as
Introduction
Neurotoxic, hemorrhagic and myotoxic
-A major neglected disease of the 21st century
-Among the most complex and least treated common The families of venomous snakes:
medical conditions in the tropics Elapidae: cobra, krait, coral snakes
-Underreported in most countries Hydropidae: sea snakes
-Most victims see only by traditional healers Viperidae: russel’s vipers,echis carinatus
-Many victims die in villages or during transport to health
care

Features of Poisonous Snakes

-A pair of poison apparatus
Snake bites in Nepal -Two fangs
-Snake bites are an important health problem in Nepal -Tail is compressed
where 80% of the population live in the rural area -Transverse ventral shield
-77 species of snakes in Nepal, 22 species are poisonous -Smaller head scales
-In the southern Terai plain (Low-land), cobras and kraits -Large infralabial shield
are the most dangerous species encountered (neurotoxic -Two bite marks
venom) -Nocturnal habit
-Access to life saving anti-venoms and facilities to Examples: Cobra,Krait,coral snake and pit viper
administer it is limited

Features of Non- Poisonous Snakes

Referral System in Health Structure of Nepal -Scales are smaller, rounded,and scales on the dorsal and
-Sub-Health-post ventral surface are exactly similar
-Primary Health Care Center -Scales on head are large, round
-District Hospital Examples: blind snakes, Boas and pythons, rat snake
-Zonal Hospital etc…
-Tertiary Care Center

SHP first contact point :

- No doctor, No Antivenom Pathophysiology of Ophitoxaemia
-Saliva
Primary Health Center: -Snake venom is the mixture of enzymatic, non
- 90% no doctor, No Antivenom (few exceptions) enzymatic compound along with other non-toxic proteins
including CHO and metals
District hospital:
-Over 20 different enzymes :
- >50% hospital has doctors
phospholipase A2, B,C,D
- Many will not treat snakebite cases
hydrolase, phosphatases,proteases,esterases
- Antivenom available, if snakebite is treated
hyaluronidases, acetylcholinesterases, ATPase
Zonal Hospital: nucleosidase & nucleotidases
- Antivenom Available -Non enzymatic components : neurotoxins and
- Some do not treat snakebite victims – e.g. Mechi hemorrhages.
- Emergency mostly attended by HA -Venoms disrupts the normal cellular function .

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Ophitoxaemia HISTORY
-Increase capillary permeability – loss of blood and -History may not be always available
plasma volume into the extra vascular space – edema & -If present ask
shock.
-Direct cytolytic action -When
local necrosis -What was done
secondary infection -Confirmed/ non confirmed
-Hemmorrhagins-damages endothelial lining
-C/O vomiting, bleeding, decreased urine, colour of urine
-Neurotoxic action : paralysis and respiratory arrest
, sleepiness, drooping eyelids , double vision,respiratory
-Cardiotoxic effect /Myotoxic & nephrotoxic
difficulty etc
effect/coagulopathy
-Have a high index of suspicion

Snake wise venom and fatal dose

LOCAL FEATURES
Authors Snake Maximum Fatal dose
-fang marks
speices extractable (mg)
-local pain
dose (mg)
-local bleeding
-bruising
Sharma King cobra 500 15-20
-lymphangitis (raised red lines tracking up the bitten
&Kumari
limb)
(1999)
-lymph node enlargement
Krait 8-12 2-3 -inflammation (swelling, redness, heat)
-blistering
Russell’s 130-250 40-70 -local infection, abscess formation
viper -necrosis

Diagnosis FANG MARKS

CLINICAL FEATURES
-A proportion of bites do not result in the injection of
venom- dry bites
-About 50% of bites by Malayan pit vipers and Russell’s
vipers, 30% of bites by cobras and 5-10% of bites by saw-
scaled vipers are dry bites.
-This might be due to mechanical factors or control of
snake on venom. SYSTEMIC FEATURES
-PTOSIS
-Even when venom is not injected
-BLEEDING- GINGIVAL SULCI
Anxiety symptoms-hyperventilation with pins and
needles, dizziness, palpitation

-Agitation and irrationality

-Vasovagal attacks
-Results of traditional caring methods

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CLINICAL FEATURES-SYSTEMIC SYNDROME 4
-Paralysis with minimal or no local envenoming
General -Bitten on land while sleeping on the ground = krait
-Nausea, vomiting, malaise, abdominal pain, weakness, -Bitten in the sea, estuary and some freshwater lakes =
drowsiness, prostration. sea snake

Cardiovascular (Viperidae)
-Visual disturbances, dizziness, faintness, collapse, shock,
hypotension, cardiac arrhythmias, pulmonary oedema, SYNDROME 5
conjunctival oedema (chemosis), bleeding -Paralysis with dark brown urine and acute kidney injury:
-Bitten on land (with bleeding/clotting disturbance) =
Neurological (Elapidae, Russell’s viper) Russell’s viper, Sri Lanka or South India
-Drowsiness, paraesthesiae, abnormalities of taste and
smell, “heavy” eyelids,
-ptosis , external ophthalmoplegia , paralysis of facial
-muscles and other muscles innervated by the cranial MANAGEMENT STEPS
nerves, nasal voice or -First aid treatment
-aphonia, regurgitation through the nose, difficulty in -Transport to hospital
swallowing secretions, -Rapid clinical assessment and resuscitation
-respiratory and generalised flaccid paralysis. -Detailed clinical assessment and species diagnosis
-Investigations/laboratory tests
-Antivenom treatment
-Observing the response to antivenom
SYNDROME 1 -Deciding whether further dose(s) of antivenom are
Local envenoming (swelling etc.) with bleeding/clotting needed
disturbances = -Supportive/ancillary treatment
Viperidae (all species) -Treatment of the bitten part
-Rehabilitation
-Treatment of chronic complications

SYNDROME 2
FIRST AID
-Local envenoming (swelling etc.) with bleeding/clotting
-Reassure the victim who may be very anxious
disturbances, shock or acute kidney injury = Russell’s
-Immobilize the whole of the patient’s body by laying
viper (hump-nosed pit viper in Sri Lanka and SW India)
him/her down in a comfortable and safe position and,
-with conjunctival oedema (chemosis) and acute pituitary especially, immobilize the bitten limb with a splint or
insufficiency =Russell’s viper, Myanmar sling. Any movement or muscular contraction increases
absorption of venom into the bloodstream and
-with ptosis, external ophthalmoplegia, facial paralysis lymphatics
etc and dark brown urine= Russell’s viper, Sri Lanka and -If the necessary equipment and skills are available,
South India consider pressure-immobilization ( craepe’s bnadage-4.5
m long by 10 cm wide)- for ?elapidae

SYNDROME 3
-Local envenoming (swelling etc.) with paralysis = cobra
or king cobra

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FIRST AID INVESTIGATIONS
-Start from bite proximally
-Allow one finger insertion in between the layers 20-minute whole blood clotting test (20WBCT)
-Do not obliterate the distal pulses -Place 2 mls of freshly sampled venous blood in a small,
-Transport to definite treatment centre immediately new or heat cleaned, dry glass vessel.
-Do not remove tourniquet immediately -Leave undisturbed for 20 minutes at ambient
temperature.
-Tip the vessel once.
-If the blood is still liquid (unclotted) and runs out, the
FIRST AID : “DONTS” patient has hypofibrinogenaemia (“incoagulable blood”)
-Interfere with bite as a result of venom-induced consumption coagulopathy
-Suck by mouth (Fig. 61).
-Apply chemicals or electricity -In the South-East Asia region, incoagulable blood is
-Use arterial tourniquets diagnostic of a viper bite
-Cooling agents on the bite -and rules out an elapid bite.
-Make incision on the wound -If the vessel used for the test is not made of ordinary
-Give anything to eat or drink glass, or if it has been cleaned with detergent, its wall
may not stimulate clotting of the blood sample (surface
activation of factor XI – Hageman factor) and test will
DO RIGHT be invalid
-R =Reassure the patient. Only 50% of bites by -If there is any doubt, repeat the test in duplicate,
venomous species actually envenomate the patient including a “control” (blood from a healthy person such
-I =Immobilise the limb in the same way as for a fracture. as a relative)
Use bandages or cloth to hold the splints, not to block
20 minute whole blood clotting test (20WBCT)
the blood supply or apply pressure
• 2-3 ml venous blood into
-G. H. =Get to Hospital Immediately. Traditional
new, clean, dry, glass vessel
remedies have NO PROVEN benefit in treating snakebite
• Leave for 20 min
-T=Tell the doctor of any systemic symptoms that
• Tip once
manifest on the way to hospital
• If blood runs out like water
= consumptive coagulopathy

HOSPITAL MANAGEMENT INVESTIGATIONS

-Airway -Hb
-Breathing (respiratory movements) -Total blood counts including platelets
-Circulation (arterial pulse) -Renal function tests
-Disability of the nervous system (level of consciousness) -Urine routine, microscopy
-Exposure -ECG
-Imaging if necessary
-Tests for secondary trauma/ co morbidities
-ABG

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POLYVALENT ANTIVENOM -Supporting laboratory evidence of systemic envenoming
-Indian cobra, Naja naja
-Indian krait, Bungarus caeruleus
-Russell’s viper, Daboia russelii
Local envenomation
-Saw-scaled viper, Echis carinatus
- Local swelling involving more than half of the bitten
limb (in the absence of a tourniquet) within 48 hours of
the bite. Swelling after bites on the digits (toes and
especially fingers).
-Rapid extension of swelling (for example, beyond the
wrist or ankle within a few hours of bite on the hands or
feet).
-Development of an enlarged tender lymph node
draining the bitten limb

ANTIVENOM
-USE AS EARLY AS POSSIBLE
-WORKS EVEN IF DELAYED PRESENTATION IN SYSTEMIC
TOXICITY
-FOR LOCAL ENVENOMATION AND NECROSIS IT WORKS
IF GIVEN WITHIN HOURS
Polyvalent ASV
Each ml. of Polyvalent ASV neutralizes
– 0.6mg of cobra venom
– 0.6mg of viper venom PREPARING TO GIVE ANTIVENOM
– 0.45 mg of krait venom -Keep adrenaline and other drugs / equipments for
– 0.45 mg of saw scaled viper venom resuscitation ready.
-Use intravenous as far as possible
-Remember to weigh the benefits and the harms of using
the antivenom
INDICATIONS OF ANTIVENOM -Always monitor
-Talk about probable effects of antivenom.
Systemic envenomation
-Haemostatic abnormalities: Spontaneous systemic
bleeding (clinical),coagulopathy (20WBCT or other
laboratory tests such as prothrombin time) or GIVING ANTIVENOM
thrombocytopenia (<100 x 109/litre or 100 000/cu mm) -Reconstitute in 10ml of water
-Neurotoxic signs: ptosis, external ophthalmoplegia, -Do not give more than 2ml/min
paralysis etc (clinical) -100 ml over an hour then 50ml 6 hourly would be
-Cardiovascular abnormalities: hypotension, shock, adequate for all (saw scaled 50 ml)
cardiac arrhythmia (clinical), abnormal ECG

-Acute kidney injury (renal failure): oliguria/anuria

(clinical), rising blood creatinine/ urea (laboratory) Criteria for giving more antivenom
-(Haemoglobin-/myoglobin-uria:) dark brown urine -Persistence or recurrence of blood incoagulability after 6
(clinical), urine dipsticks,other evidence of intravascular hours or of bleeding after 1-2 hours
haemolysis or generalised rhabdomyolysis(muscle aches -Deteriorating neurotoxic or cardiovascular signs after 1-
and pains, hyperkalaemia) (clinical, laboratory) 2 hours

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Protocol: a. Early anaphylactic reactions:
-Usually within 10-180 minutes
National – 2vials + 4 vials/4hourly X2-3 +
2vials/4hourlyX2-3+ 2vials/6hourly till symptoms of -What do you expect?
envenoming disappears -What will you instruct?
-What will you do ?
International : Initial dose – 10 vials iv push/drip repeat
(half or full dose) if clinical deterioration of neurotoxicity. ANAPHYLAXIS MANAGEMENT
-Inj adrenaline 0.5 mg im
-Inj hydrocortisone 100mg or 2 mg/ kg
-Inj antihistamine (H1) iv
-Oxygen – high flow
ANTI VENOM EFFECTS -Bronchodilators
-General: The patient feels better. Nausea, headache and -Intubate if necessary
generalised aches and pains may disappear very quickly. -Intravenous fluids

- Spontaneous systemic bleeding (e.g. from the gums):

This usually stops within 15-30 minutes.
b. Pyrogenic (endotoxin) reactions:
- Blood coagulability (as measured by 20WBCT): This is -Usually 1 to 2 hours after treatment
usually restored in 3-9 hours. Bleeding from new and -Symptoms include shaking chills (rigors), fever,
partly healed wounds usually stops much sooner than vasodilatation and a fall in blood pressure.
this. -Febrile convulsions may be precipitated in children.
-These reactions are caused by pyrogen contamination
- In shocked patients: Blood pressure may increase within during the manufacturing process.
the first 30-60 minute and arrhythmias such as sinus -Treat similar to anaphylaxis-adr,steroids,anti histamine
bradycardia may resolve.

- Neurotoxic envenoming of the post-synaptic type

(cobra bites) may begin to improve as early as 30 c. Late (serum sickness type) reactions:
minutes after antivenom, but usually takes several hours. -Usually 1 to 12 days (7) days after treatment.
Envenoming with presynaptic toxins (kraits and sea -Clinical features include fever, nausea,vomiting,
snakes) will not respond in this way. diarrhoea, itching, recurrent urticaria, arthralgia,myalgia,
lymphadenopathy, periarticular swellings, mononeuritis
- Active haemolysis and rhabdomyolysis may cease multiplex, proteinuria with immune complex nephritis
within a few hours and the urine returns to its normal and, rarely, encephalopathy.
colour. -Treat with antihistamines and corticosteroid

OTHERS
-Neostigmine controversial
ANTI VENOM REACTIONS
-May be tried for cobra bite with neurotoxicity
-usually more than 10%
-Neostigmine 0.02 mg/kg adults, 0.04 mg / kg children
-risk of reactions is dose-related, except in rare cases in
-Give atropine before giving neostigmine
which there has been sensitization (IgE-mediated Type I
hypersensitivity) by previous exposure to animal serum,
for example, to equine antivenom, tetanus-immune
globulin or rabies-immune globulin

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COMPARTMENTAL SYNDROME Species: Medical Implications

Clinical features of a compartmental syndrome Signs/Symptoms Cobr Krai Russell Saw Othe
-Disproportionately severe pain and Potential a t ’s Scale r
-Weakness of intracompartmental muscles Treatments Viper d Viper
-Pain on passive stretching of muscles Viper s
-Hypoesthesia of areas of skin supplied by nerves running
through the compartment Local pain/ Yes No Yes Yes Yes
-Obvious tenseness of the compartment on palpation Tissue Damage

Ptosis/Neurotoxi Yes Yes Yes! NO No

city
FASCIOTOMY
Criteria for fasciotomy in snake-bitten limbs Coagulation No No Yes Yes Yes
-Haemostatic abnormalities have been corrected
Renal Problems No No Yes NO Yes
(antivenom with or without clotting factors)
-Clinical evidence of an intracompartmental syndrome Neostigmine & No
Yes No? NO No
-Intracompartmental pressure >40 mmHg (in adults)
Atropine ?
-Controversial whether to perform or not

SUPPORTIVE
-Oxygen if required
-Pain relief
-Injection Tetanus Toxoid
-Fluid challenge / diuretics / dialysis
-Intubation
-Blood Transfusion if necessary
-Debridement / antibiotics
-Physiotherapy

Prevention of Snake Bites

-The snake is almost always more scared of you than you
are of the snake. Giving the snake the opportunity to
escape prevents most bites.
-Do not attempt to handle, capture, or tease.
-Snakebites are often associated with alcohol use.
-For outdoors activities- wearing boots while hiking, Long
pants can reduce the severity of a bite,
-Be cautious where you place your hands and feet (for
example, when gathering firewood or collecting berries),
and never walk barefooted after dark.
-If your occupation or hobby exposes you to dangerous
snakes on a regular basis, preplanning is necessary.

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