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12+rheumatology Drawio

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22 views5 pages

12+rheumatology Drawio

Summary.nots

Uploaded by

Abdelhady Ali
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Contents

10. Haematology & Oncology


10.01-02 Haematological cancers 15. Paediatrics / Child Health
10.03 Primary immunode iciencies, Acute intermittent 15.01 Paediatric life support
porphyria 15.02-03 Infections
10.04-06 Anaemias 15.04-06 Cardiovascular disorders
10.07 Platelet disorders, Neutropaenia 15.07-09 GI disorders

f
10.08 Blood products 15.10-11 Renal & urological disorders
10.09 Haematology – miscellaneous 15.12-13 Neurological disorders
10.10 Haematological emergencies, Organ transplants 15.14-15 Haemonc disorders 15.16-18
10.11 Oncology – miscellaneous Endocrine disorders
10.12 Breast pathology 15.19 Developmental disorders
15.20-21 Genetic disorders
11. Orthopaedics & Musculoskeletal problems 15.22-23 Neonates
11.01 Orthopaedic emergencies 15.24 NAI, screening, vaccinations
11.02 Upper limb orthopaedic issues
11.03 Upper limb orthopaedic issues, Foot 16. Obstetrics
11.04 Lower limb orthopaedic issues – hip 16.01-03 Antenatal care
11.05 Lower limb orthopaedic issues – knee and ankle 16.04-06 Pre-existing problems in pregnancy
09.06 Spinal orthopaedics 16.06-07 Ante/perinatal infections
09.07-08 Orthopaedics – miscellaneous conditions 16.08 Antenatal problems
09.09 Orthopaedic malignancies 15.09 Perinatal baby problems
16.10-13 Labour and problems
12. Rheumatology 16.14-15 Obstetric emergencies
12.01 RA, OA, Psoriatic arthritis 16.16-17 (Obs/Gynae) Contraception
12.02 Rheumatic conditions (muscular problems)
12.03 Rheumatic conditions (with skin problems) 17. Gynaecology
12.04 Vasculitis 17.01 FGM, Amenorrhoea, PCOS
17.02 Menorrhagia, PMS, Menopause, HRT
13. ENT 17.03 Termination of pregnancy, Miscarriage
13.01-03 Ear 17.04 Ectopic pregnancy, Gestational trophoblastic disease
13.04 Nose 17.05 PID, Endometriosis, Fibroids
13.05-07 Throat 17.06 Subfertility, OHSS
17.07 Vulval problems
14. Psychiatry 17.08 Vaginal discharge, Urinary incontinence, Pelvic organ prolapse
14.01 Mood disorders 17.09 Cervical cancer + screening, Vaginal cancer, Endometrial cancer
14.02 Anxiety & related disorders 17.10 Ovarian cancer, cysts, torsion
14.03 Psychotic disorders
14.04 Personality disorders
14.05 Eating disorders & miscellaneous psychiatric disorders
14.06 Drugs & MHA/Sectioning

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Rheumatoid arthritis Osteoarthritis Psoriatic arthritis

D: chronic, erosive arthritis. Affects ~1% of (Mx cont) DMARDs D: degenerative joint disorder, the result of Mx: surgical - joint replacement D: chronic inflammatory joint disease
population. • MTX - monitor FBC, LFTs mechanical and biological events that • For pts with significant pain associated with psoriasis. Oligo or mono-
- AE: myelosuppression, liver failure, destabilise the normal process of • Hips: cemented hip replacement, articular initial pattern + DIP
R: F>M, FHx (main), ?smoking pneumonitis degradation and synthesis of articular uncemented HR (in younger, more active
• Sulfasalazine cartilage chondrocytes, extracellular matrix, pts; more expensive), hip resurfacing R: psoriasis, FHx of psoriasis or PA
A/P: Inflamed synovium → infiltration of - AE: rashes, oligospermia, heinz body and subchondral bone (preserves femoral neck)
Exacerbating factors: trauma, alcohol,
inflammatory cells + cytokines → erosions anaemia, ILD (lung) • Post-op: physio, home-exercises, walking
drugs (βBs, lithium, antimalarials, NSAIDs,
• Leflunomide R: >50yo, F>M, obesity, genetic factors, sticks / crutches for ≤6w
- AE: liver failure, interstitial lung disease, knee alignment, ↑activity, post-trauma • How to avoid dislocation: avoid flexing hip ACEI, infliximab), withdrawal of steroids.
Earlier s/smx: HTN >90', avoid low chairs, do not cross legs, Strep infxn for guttate psoriasis
• Symmetrical arthritis >6w (insidious
development; small → large joints)
• Hydroxychloroquine S/smx: pain, functional difficulties, ↓ROM, sleep on back for first 6w
- AE: Retinopathy, corneal deposits malalignment. Knee > hip > hand Mx: conservative + medical A/P: likely genetic, a/w HLA B27. Sero-
• Swollen, painful joints in hands/feet • Gold – AE: Proteinuria • wt loss, muscle strengthening negative. CD8 T-cells eat away at the
• Stiffness worse in the morning • Penicillamine – AE: proteinuria, ↑MG OA of the hip • non-Rx: supports, braces, TENS, shock- joints.
• ± systemic disturbances • chronic hx of groin ache; ↑by exercise, absorbing insoles/shoes
TNFα inhibitors ↓by rest - "C sign" •☝🏻 paracetamol, NSAIDs
✌🏻
Later s/smx: > indicated if inadequate response to ≥2 S/smx:
• Oxford Hip Score to Ax severity • Opioids, capsaicin cream, intra-
• Deformities: swan neck, boutonniere, DMARDs including MTX • Symmetric polyarthritis (similar to RA)
• Etanercept = recombinant protein, acts articular corticosteroids • or asymmetrical oligoarthritis (hands and
Z-thumb, ulnar deviation OA of the hand - PPI must be prescribed with NSAIDs
• ?+ve FHx, F>M, >55yo, ↑risk for hip,
• +ve squeeze test as decoy receptor for TNF-α feet – less common than above)
- SQ admin • Sacroiliitis
knee OA
• R: hypermobility, ↑working with hands
Others: relapsing/remitting monoarthritis of - AE: demyelination, reactivation of TB Complications: • DIP joint disease (10%)
different large joints (palindromic • Infliximab = (mab) binds to TNF-α and • VTE, fracture, nerve injury, surgical site • Athritis mutilans (severe deformity of
• Usually bilateral, basal thumb joint
rheumatism) prevents it from binding TNF receptors infxn; leg-length discrepancy hands, 'telescoping fingers')
(CMC), DIPs
- IV admin • Posterior dislocation - presents acutely • Nail changes (80-90%): pitting,
• Episodic joint pain (intermittent), stiffness
- reactivation of TB with clunk, pain, inability to wt bear. O/E onycholysis, subungual hyperkeratosis,
Dx: clinical + lab confirmation (short)
• Adalimumab: (mab) SQ admin internal rotation, shortening of affected leg loss of nail
• Rheum screen: RF (70%), anti-CCP • painless nodes - Hebedern's (DIP),
• Aseptic loosening (most common reason ± Psoriatic skin lesions
(90-95%) Rituximab Bouchard's (PIP) - 2/2 osteophytes
for revision) + prosthetic joint infxn ± Periarticular disease
• X-rays of hands & feet (NICE rec) • anti-CD20 mab, ↓B cell • Squaring of the thumbs (causes fixed
- Enthesitis
- early: loss of joint space, juxta-articular • two 1g IV infusion, 2 weeks apart adduction of thumb)
- Tenosynovitis (esp hand flexors)
osteoporosis, soft-tissue swelling • AE: infusion reactions 🚩 rest pain, night pain, morning stiffness - Dactylitis - uniquely psoriatic arthritis
- periarticular erosions, subluxation
Abatacept (not recommended by NICE) >2h → ?alt cause
• Fusion protein that modulates key signal Ix: XR - erosive Δs, new bone formation,
Am College of Rheumatology criteria
• joint involvement required for activation of T-cells; ↓T cell Ix: XR of affected joint - LOSS periostitis, 'pencil in cup' appearance
- more points for more joints prolif, ↓cytokines • Loss of joint space Bloods - ESR, CRP, RF, anti-CCP
- ≥1 small joint must be involved • IV infusion • Osteophyte formation
• serology (+ve ↑↑RF, anti-CCP) • Subchondral cysts & sclerosis Mx: Tx as for RA
• acute phase reactants (↑CRP, ESR) P: following a/w ↓prognosis r/o RA if needed (FBC, RF, anti-CCP) • with mild arthritis, NSAID monotherapy
• duration of smx (>6w) • RF • anti-CCP • HLA-DR4 may be sufficient
• poor functional status at presentation • Ustekinumab and secukinumab
Mx: • X-ray: early erosions (<2y)
• Extra-articular features P: better than RA
• Start DMARD asap!!
- monotherapy ± short-course of • Insidious onset • ?F sex
bridging prednisolone
• Monitoring: CRP + DAS28 score Complications / extra-articular manifestations
• Ocular manifestations in 25%: keratoconjunctivitis sicca,
Flares: • Corticosteroids PO/IM episcleritis, slceritis, corneal ulceration, keratitis
• Respi: pulmonary fibrosis, pleural effusion, pulmonary
nodules, bronchiolitis obliterans, pleurisy
• Osteoporosis (2/2 steroids?)
• Ischaemic heart disease
• ↑risk of infections (2/2 DMARDs?)
• Depression • Amyloidosis
• Felty's syndrome: RA + splenomegaly + neutropenia
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12.01 Rheumatology – RA, OA, Psoriatic arthritis
12.02 Rheumatology – Rheumatic conditions (muscular problems)

Ankylosing spondylitis Gout Pseudogout Polymyalgia rheumatica Chronic fatigue syndrome


more accurately acute calcium
D: chronic progressive inflammatory D: mono/oligo-articular inflammatory pyrophosphate crystal deposition disease D: an inflammatory rheumatologic aka myalgic encephalomyelitis
arthropathy of primarily the axial skeleton microcrystal synovitis syndrome. A/w GCA in some pts D: Condition characterised by disabling
D: microcrystal synovitis caused by the fatigue affecting mental and physical
R: FHx (97% hereditability), HLA-B27, A/P: chronic hyperuricaemia deposition of calcium pyrophosphate R: ≥50yo, GCA, ?F>M function more than 50% of the time for
ERAP1 and IL23R genes, young M>F (>450umol/L) ↑risk of crystal formation dihydrate crystals in the synovium at least 3 mo
A/P: unknown / unclear • Smx not better attributed to other
New York criteria for Dx R: ↑age (>40), M>F, menopause, R: ↑age. RF for pseudogout at <60yo: disorder
1. low back pain ≥3mo, improved by consumption of meat, seafood, alcohol, haemochromatosis, hyperPTH, ↓Mg, S/smx
↓PO4, acromegaly, Wilson's disease R: F>M, lower socioeconomic status,
exercise, not relieved by rest Rx (diuretics, ciclosporin, tacrolimus, • Rapid onset (<1mo)
• Aching, morning stiffness in proximal
↑BMI, ↑physiological toll (eg pregnancy,
pyrazinamide, aspirin), FHx, ↑cell

• may come on at night; improves on inadequate rest), chronic health
getting up turnover rate S/Smx: painful and tender joints. knee, limbs ( weakness) conditions (eg DM, cancer),
2. limited lumbar spinal motion wrist and shoulders most commonly • Others: mild polyarthralgia, lethargy,
FHx – Lesch-Nyhan syndrome (enzyme psychological stress (eg depression)
• Schober's test – ↓forward flexion deficiency, X-linked recessive [M>>F], with
affected. Chronic form mimics OA/RA, depression, low-grade fever, anorexia,
3. ↓chest expansion for age/sex gout, renal failure, neuro deficits, learning
a/w variable degrees of inflammation. night sweats
Cause & pathology not well understood
4. XR evidence of sacroiliitis difficulties, self-mutilation)
• bilateral grade 2 to 4 Ix: arthrocentesis with synovial fluid Ix: ↑ESR (>40). Normal CK and EMG. S/smx: • Tiredness/fatigue
• or unilateral grade 3 to 4 S/Smx: 70% in first MTP, rapid onset analysis [weakly-positively birefringent
Passmed's – other features 'A's severe pain, joint stiffness, swelling and rhomboid-shaped crystals], XR • Sleep problems – ↑ or ↓ sleep
Mx: prednisolone (e.g. 15 mg OD) - pt • Myalgia, arthralgia
• Apical fibrosis • Anterior uveitis joint effusion, tenderness ± tophi [chondrocalcinosis]
usually responds dramatically and w/in 24h. • HA • Painful LN
• Aortic regurg • Achilles tendonitis If no response, consider other dx.
• AV node block • Amyloidosis Mx: arthrocentesis (exclude septic • Sore throat • "Flu-like" smx
Ix: synovial fluid analysis [needle shaped • continue until ESR/CRP resolves • Cognitive dysfunction (eg inability to
• Cauda equina • Peripheral arthritis negatively birefringent MSU crystals arthritis), NSAIDs ± intra-articular, intra-
• Taper slowly. Tx usually ~1y concentrate)
under polarised light], serum uric acid muscular or PO steroids as for gout
• Dizziness, nausea
Ix: XR – ∆ apparent only in later disease levels [check after 2w after acute • Palpitations
• Sacroiliitis: subchondral erosions, episode], XR
erosions
Ix – to r/o other conditions, esp cancer
• Squaring of lumbar vertebrae ACUTE Mx Reactive arthritis Fibromyalgia
☝🏻
• "Tired all the time" (TATT) bloods: FBC,
• 'Bamboo spine' – late, uncommon NSAIDs, colchicine U&Es, LFTs, TFTs, ESR/CRP, HbA1c,
• Syndesmophytes 2/2 ossification of • colchicine has slower onset, also D: inflammatory arthritis that occurs after D: chronic pain syndrome diagnosed by the IgA TTE, CK, bone profile + consider Vit
annular fibrosus causes diarrhea
✌🏻
exposure to certain GI and GU infections presence of widespread body pain D, Vit B12 & folate, iron studies,
• CXR - apical fibrosis PO steroids (e.g. prednisolone 15
one of the HLA-B27 associated seronegative monospot test, HIV test, hepatitis
mg/d) or intra-articular steroids spondyloarthropathies R: FHx, rheumatological conditions, 20- serology, early morning cortisol
If XR -ve, MRI may capture earlier
changes
** if pt is already on allopurinol, continue 60yo, F>M ↳ CK will be normal
A/P: a/w Shigella, Salmonella, Yersinia, • Other tests: urine dip, CXR, sputum
Spirometry – restrictive defect Indications for urate-lowering therapy
Campylobacter, Chlamydia. ?triggered by samples for TB
** after first attack of gout A/P: CNS – pain or sensory amplification;
bactierial DNA in synovial tissue "nociplastic pain". Centralisation of pain.
Mx: • regular exercise, physio esp if ≥2 attacks in 12mo, tophi, renal
• ☝🏻 NSAIDs disease, uric acid renal stones,
Mx: • Refer to specialist CFS service
• ✌🏻 anti-TNFα drugs if refractory prophylaxis if on cytotoxics or diuretics R: M>F, HLA-B27, preceding chlamydial
or GI infection
S/smx: • hyperalgesia, allodynia
• Energy Mx: help with managing energy
levels to continue functioning
• DMARDs only useful if peripheral joint • Multiple sites; "pain all over" • Physical activity & exercise
involvement PROPHYLACTIC Mx
☝🏻
• Lethargy, cognitive impairment, 'fibro fog', - Under advice of ME/CSF specialist
Allopurinol 100 mg OD, titrate to uric S/smx: "cannot see, cannot pee, cannot sleep disturbances, HA, dizziness team
acid <300 umol/L climb a tree" (urethritis, conjunctivitis, - Graded exercise therapy not
? start after acute episode is over arthritis) recommended by NICE
Dx is clinical; normal bloods.
+ colchicine / NSAID cover (≤ 6mo)
✌🏻 febuxostat Ix: Hx taking, bloods (ESR, CRP, ANA, Mx: bio-psycho-social model
• Cognitive behavioural therapy
3rd: (refractory) uricase, IV pegloticase RF, ?HLA-B27). • Pt education on condition • CBT
• Aerobic exercise (most evidence)
Mx: symptomatic - analgesia, NSAIDs, • Pregabalin, duloxetine, TCAs
intra-articular steroids
persistent disease - sulfasalazine, MTX

P: smx rarely last >12mo


© qqm/jmsn
Systemic lupus erythematosus Systemic sclerosis ≈ scleroderma Dermatomyositis Sjogren syndrome

D: chronic multisystem disorder D: multi-system, autoimmune disease, D: idiopathic autoimmune inflammatory D: chronic inflammatory and auto- Ix: RF 50%, ANA 70%, anti-Ro
characterised by presence of ANA characterised by functional and structural myopathy characterised by distinctive skin immune disorder characterised by (SSA) 70%, anti-La (SSB) 30%,
abnormalities of small blood vessels, manifestations diminished lacrimal and salivary Schirmer's test – filter paper near
R: F>M, >30yo, African descent in fibrosis of skin and internal organs, and gland secretion conjunctival sac to measure tear
Europe/US & Asians, drugs production of auto-abs R: FHx, children or >40yo, F>M, black formation. Histology: focal
(sulfasalazine, isoniazid, phenytoin, ethnicity, UV radiation R: F>M, SLE, RA, scleroderma, HLA lymphocytic infiltration. ±
carbamazepine) R: FHx, immune dysregulation (e.g. +ve May be a/w underlying malignancy II markers, 20-30yo or after hypergamma-globulinaemia, low C4
ANA), F>M (4:1) (typically ovarian, breast, lung) – screen for menopause
A/P: type III HS rxn, a/w HLA B8, DR2, underlying cancer Mx: artificial tears and saliva
DR3. 🤔 immune system dysregulation LeRoy classification and features: A/P: immunological, genetic, ± pilocarpine to stimulate saliva
→ immune complex formation → • Diffuse cutaneous systemic sclerosis: A/P: autoimmune; autoantibodies present, hormonal, and viral components ±
affects any organ skin thickening on the proximal evidence of T-cell mediated muscle injury, estrogen involvement P: ↑↑ lymphoid malignancy (40-60x)
extremities or the trunk + face and distal complement-mediated vascular damage
S/smx: RASH or PAIN extremities S/smx
• Rash: malar, discoid - a/w Scl-70 antibodies S/smx – myositis • dry eyes: keratoconjunctivitis sicca
- discoid: scaly, red, well-demarcated - respi involvement – 80% interstitial lung • Proximal muscle weakness ± tenderness • dry mouth
rash in sun-exposed areas disease, pulmonary artery HTN (most • Raynaud's • vaginal dryness • arthralgia
• Arthritis, athralgia common cause of death) • Respiratory muscle weakness • Raynaud's, myalgia
• Serositis: pleuritis, pericarditis - also renal disease, HTN • Interstitial lung disease • sensory polyneuropathy
• Haematologic: cytopenias, ITP, etc - poor prognosis :( • Dysphagia, dysphonia • recurrent episodes of parotitis
Antiphospholipid syndrome
• Oral, nasopharyngeal ulcers • Limited cutaneous systemic sclerosis: S/smx – skin lesions • RTA (usually subclinical) → See 10.06
• Renal disorders, Raynaud's skin thickening confined to sites distal to • Photosensitive
• Photosensitivity the elbows and knees ± face • Macular rash over back, shoulder
• ANA+ve - a/w anti-centromere abs • Periorbital heliotrope rash
• Immunoglobulins – anti-dsDNA, - CREST: calcinosis, Raynaud's, • Gottron's papules Eosinophilic
Granulomatosis granulomatosis
anti-Smith, antiphospholipids esophageal dysmotility, sclerodactyly, • Mechanic's hands – extremely dry and Wegener's with polyangiitis
Churg-Strauss
• Neurologic: seizures, psychosis telangectasia scaly hands with linear cracks with polyangiitis
• Sine scleroderma: no skin involvement • Nail fold capillary dilatation • D: autoimmune condition a/w a D: autoimmune condition associated
• 'Pure' scleroderma: no internal organ necrotizing granulomatous vasculitis, with a necrotizing granulomatous
Ix: autoimmune screen (99% ANA, 70% involvement
anti-dsDNA, 30% anti-Smith, etc). ESR Ix: ↑↑CK, serum aldolase, muscle and affecting both the upper and lower vasculitis, affecting the respiratory
- Tightening and fibrosis of skin
monitored; ↑CRP may indicate infection. - Plaques (morphea) or linear
skin biopsy (vasculitis), EMG abn, 70% respiratory tract and kidneys tract (?only)
↓complement levels during active ANA+ve. Remainder have abs to anti-Jo-1, • S/smx
disease. ? anti-dsDNA titres to monitor anti-SRP, anti-Mi2 - URT: epistaxis, sinusitis, nasal crusting S/smx
disease development if +ve Ix: serum auto-abs, full bloods, ESR, - LRT: dyspnoea, haemoptysis
CRP, pulmonary function tests, ECG, echo, • asthma
Mx of acute flares: - Rapidly progressive glomerulonephritis
☝🏻 CXR, barium swallow, etc • blood eosinophilia (e.g. > 10%)
Mx: NSAIDs, sunblock, HCQ! • IV corticosteroids ± IVIG nephritis ('pauci-immune', 80% of • paranasal sinusitis
If internal organ involvement, • DMARD, rituximab cases)
🤔
• mononeuritis multiplex
Mx: according to smx (e.g. ACEI for renal - Saddle-shape nose deformity
✌🏻
prednisolone, cyclophosphamide Mx of ongoing disease: PO steroids • pANCA positive in 60%
disease, CCB for Raynaud's). DMARDs • sun protection - Also vasculitic rash, eye involvement
for advanced/severe disease • topical steroids for skin lesions ± tx (e.g. proptosis), CN lesions
malignancy • Ix
Lupus nephritis P: mean survival ~12y after Dx - cANCA >90%, pANCA in 25%
WHO classification - CXR: wide variety
• class I: normal kidney - Renal biopsy: epithelial crescents Wegener's
• II: mesangial glomerulonephritis Mx: • Treat HTN Polymyositis
in Bowman's capsule
• III: focal, segmental proliferative GN • Initial therapy for class III and IV: • Mx: - steroids renal failure vasculitis asthma
• IV: diffuse proliferative GN glucocorticoids + mycophenolate or D: idiopathic autoimmune inflammatory - cyclophosphamide epistaxis sinusitis eosinophilia
- most common, severe cyclophosphamide myopathy characterised by only muscle - Plasma exchange haemoptysis dyspnea pANCA
- 'wire-loop' appearance, thickened • Subsequent therapy: mycophenolate, inflammation • P: median survival 8-9y cANCA
capillary wall, immune complex azathioprine Similar to dermatomyositis apart from Churg-Strauss
deposits, granular appearance P: can result in ESKD requiring not having skin lesions
• V: diffuse membranous GN See next pg for microscopic polyangiitis
transplant. Urine dipstick to monitor,
• VI: sclerosing GN r/o proteinuria

© qqm/jmsn
12.03 Rheumatology – Rheumatic conditions (with skin problems), ANCA-associated vasculitis
12.04 Rheumatology – Vasculitis

Vasculitis Giant cell arteritis Takayasu's arteritis Polyarteritis nodosa Henoch-Schonlein purpura

D: inflammation of the blood vessels. D: granulomatous vasculitis of large and D: chronic granulomatous vasculitis D: necrotising inflammation of medium- D: IgA mediated small vessel vasculitis.
Usually refers to autoimmune systemic medium-sized arteries affecting large arteries: primarily the sized or small arteries without glomerulo- A/w IgA nephropathy.
vasculitides. ÷vessel sizes aorta and its main branches nephritis or vasculitis in arterioles,
R: >50yo, F>M, ?FHx capillaries or venules S/smx:
R: age>50, white ancestry, sex R: FHx, F>M, <40yo, Asian (Jap) • palpable purpuric rash
(depending on disorder) S/smx: R: HBV, 40-60yo, M:F 2:1 (+ localized oedema) over buttocks
• Site: branches of external carotid S/smx and extensor surfaces of arms and legs
A/P: autoimmune. Fibrinoid necrosis of artery, esp temporal artery, skip • systemic features of a vasculitis, eg • abdominal pain • polyarthritis
A/P: triggers – immune complexes, • ± IgA nephropathy: haematuria, AKI
endothelial dysfunction, etc → intimal
the vessel wall with fragmentation of lesions malaise, headache
proliferation → thrombosis → ischaemia
chromatin into unstructured granules and • Onset: <1mo • unequal BP in the upper limbs
RBC extravasation. • Character: tender, palpable temporal • carotid bruit and tenderness Mx: analgesia, supportive tx of
or infarction of organ/tissue ± aneurysm
artery + HA (85%), jaw claudication • absent or weak peripheral pulses nephropathy. inconsistent support for
(65%), ocular complications (majority • UL or LL claudication on exertion steroids and DMARDs
S/Smx: constitutional smx S/smx: • fever, malaise, athralgia, wt
anterior ischemic optic neuropathy) • aortic regurgitation (~20%)
General: abdo pain, foot drop, wrist drop, loss, HTN
- Fundoscopy: swollen pale disc • a/w renal artery stenosis P: generally good, self-limiting in
ulcers, haematuria, purpura, nasal smx, • mononeuritis multiplex,
and blurred margins
❗️
children. Monitor BP and urinalysis. 1/3
- amaurosis fugax → permanent
sinus pain, wheeze sensorimotor polyneuropathy [→04.09]
Ix: ↑ESR (>50), ↑CRP, CTA or MRA pts have relapse
blindness [→05.01]
• See specific conditions for s/smx • testicular pain
(shows segmental narrowing or occlusion See 15.14 for more details
• A/w: PMR (50% – aching, morning of affected vessels) • livedo reticularis
Ix: ESR, CRP, ANCA, U&E, urinalysis, stiffness in prox limbs) • haematuria, renal failure
biopsy of affected tissue • Also: also lethargy, depression, low- • ANCA (20%) in 'classic' PAN
Mx: prednisolone (high to low dose),
grade fever, anorexia, night sweats • Hep B serology +ve (30%)
aspirin, alendronate Behcet's syndrome
Mx: corticosteroids + bone protective
agents, cyclophosphamide Ix: ↑ESR >50, possibly ↑CRP Ix: CRP, ESR, FBC, rheum screen, etc
D: complex multisystem disorder a/w
P: relapsing disease. Cardiac failure is a
• Temporal artery biopsy presumed autoimmune-mediated
common cause of death.
P: likely lifelong Mx required - Histology: multinucleated giant cells Mx: PO prednisolone, DMARD – inflammation of the arteries and veins
? Duplex US of temporal artery: cyclophosphamide ± IVMP
hypoechoic halo sign
❗️
Large-vessel vasculitis R: eastern Mediterranean, M>F, young
• Takayasu's arteritis CK and EMG should be normal
adults (20-40yo), HLA-B51, FHx
• Giant cell arteritis
Medium-vessel vasculitis ❗️
Mx: urgent 60mg prednisolone PO Microscopic polyangiitis
S/smx:
• Polyarteritis nodosa • if no visual loss, high dose pred
• Kawasaki's disease • if visual loss, IV methylpredinsolone • classic triad: oral ulcers, genital
D: small-vessel ANCA vasculitis ulcers, anterior uveitis
Small-vessel vasculitis before PO prednisolone
• Wegener's - there should be a dramatic response • thrombophlebitis and DVT
• Microscopic polyangiitis for pts with visual smx S/smx: • arthritis • erythema nodosum
• Churg-Strauss syndrome • same-day ophthalmology review • renal: ↑Cr, haematuria, proteinuria • neurological involvement (e.g.
• Cryoglobulinaemic vasculitis • if tx with steroids, + bisphosphonate • fever, lethargy, myalgia, weight loss aseptic meningitis)
• Henoch-Schönlein purpura • ?low-dose aspirin • rash: palpable purpura • GI: abd pain, diarrhoea, colitis
• Leukocytoclastic angiitis • cough, dyspnoea, haemoptysis
Variable vessel vasculitis P: Tx usually required for 1-2y. • mononeuritis multiplex Ix: Clinical Dx.
• Cogan syndrome Early neuro complications: vision loss, • Pathergy test – needle prick →
• Behçet's disease. CVA. Late complications: relapses, CVA, Ix: pANCA 50-75%, cANCA 40% puncture site becomes inflamed with
Single organ vasculitis aortitis leading to aortic aneurysm and small pustule – suggestive
• Cutaneous leukocytoclastic angiitis aortic dissection.
• Cutaneous arteritis Mx: • prednisolone + azathioprine (or
• Primary central nervous system other DMARDs).
vasculitis • Ulcers – topical triamcinolone paste
• Isolated aortitis • VTE prevention, etc

© qqm/jmsn

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