RHEUMATIC FEVER
DR : YOUSIF ADAM ALI
MBBS - U OF G ---- MDPCH - SMSB
ASSISTANT PROFESSOR OF PAEDIATRICS & CHILD HEALTH
General considerations :
Rheumatic fever is an immunological response that follows infection of the pharynx by
group A beta-hemolytic streptococci. It affects the heart, joints, CNS, skin and subcutaneous tissue.
it occurs 1 – 5 weeks ( average 3 weeks ) after the attack of sore throat.
Commonly occurs in age group 5 – 15 years ( with a peak incidence at 8 years of age )
Incidence is equal in males and females. Mitral valve disease and chorea are common in
females , but aortic valve involvement common in males
Attack rate of rheumatic fever after streptococcal pharyngitis is 0.3 % to 3% in children who
are not treated or inadequately treated.
Important predisposing factors include: family history of Rheumatic fever (hereditary
predisposition) , low socioeconomic status , previous attack ( 50% risk) and age group
between 5 – 15 years.
PATHOGENESIS:
The mechanism is unknown but There are three proposed hypothesis by which it affects the
human system:
1. Direct infection of the heart valves by group A beta-hemolytic streptococci. Since the
streptococci have not been isolated from the lesions in the heart and joints, this hypothesis is
not proven.
2. Extracellular toxin produced by the streptococci affecting the myocardium, valves,
synovium and brain; again this is not proved.
3. An abnormal immune response generated by the individual in response to infection and the
resulting antibodies might cause immunological damage to target organ cells . This widely
accepted theory
Conti……
The immune response triggered by colonization of the pharynx with group A streptococci
consists of :
1- sensitization of B lymphocytes by streptococcal Antigen
2- formation of antistreptococcal antibodies
3- formation of immune complexes that cross- react with cardiac sarcolemma antigens
4- myocardial and valvular inflammatory response
PATHOLOGY:
The inflammatory lesion is present in the heart , joints, brain and skin.
Aschoff body in the atrial myocardium is the most characteristic feature of rheumatic fever
It is present only in acute stage and undergoes progressive fibrosis. Now believed to be
necrotic myocardial cells.
Mitral valve is the commonest valve to be affected followed by the aortic and tricuspid
valves in that order. Pulmonary valve rarely involved .
The valves on the left side are commonly involved due to the greater strain on these valves.
Rheumatic vegetations are small , present on the margins of the valves and are formed due
to aggregation of platelet thrombi, McCallum's patch is seen on the posterior wall of the left
atrium above the mitral valve.
CLINICAL FEATURES:
Symptoms occur about 1-5 weeks (average 2-3 weeks) after an initial attack of pharyngitis.
History of preceding sore throat is present in 50% of patients .
Fever, anorexia, lethargy, fatigability, pallor and joint pains may be present.
Family history of rheumatic fever is often positive
Diagnosis of acute Rheumatic fever based on revised Duckett Jone's Criteria , which is further
divided into 5 major, 4 minor and essential criteria as follows:
1. Major criteria (more specific)
(1) Polyarthritis
(2) Carditis
(3) Chorea (Sydenham's chorea)
(4) Subcutaneous nodules
(5) Erythema marginatum
Conti….
2- Minor criteria (less specific)
1 - Fever
2 - Arthralgia
3 - elevated acute phase reactant : (ESR) and C-reactive protein ( CRP)
4 - Prolonged P-R interval on ECG .
3- Essential criteria: supporting evidence of antecedent streptococcal infection
(a) Increased or rising anti streptolysin 0 (ASO) titer ( in 80 % of cases)
(b) Anti- deoxy ribonuclease B (anti-DNase B) titer-this will peak after 6--8 weeks of infection.
(c) Positive throat culture-sensitivity (25%-40%)
(d) Recent scarlet fever/streptococcal sore throat-within previous 45 days
1 - Polyarthritis:
Arthritis is most common manifestation of acute rheumatic fever it occurs in 70 % of cases
Usually involves large joints ( knees, ankles , elbows , wrists in that order)
Often more than one joint is involved, either simultaneously or in succession with
characteristic migratory nature ( migratory or fleeting polyarthritis )
The affected joint become painful, hot , red ,swollen ,tender with limitation of movement
The arthritis responds dramatically to aspirin therapy within 12-24 hours (if it does not
respond in 48 hours the diagnosis of acute Rheumatic fever is probably incorrect and
another diagnosis should be considered).
The severity of arthritis is not related to the degree of cardiac involvement.
It resolve completely without residual damage of the joints
2 - Carditis:
Carditis occurs in 50%--60% of patients
It is one of the earliest feature and develops within the first two weeks of the onset of
rheumatic fever.
It is a pancarditis and involves pericardium, myocardium and endocardium.
Signs of Active Carditis :
1- Endocarditis ( valvulitis) :
symptoms and signs of heart failure (cough , edema ,tachypnea, tachycardia , tender hepatomegaly)
Panystolic murmur of mitral regurgitation
Early diastolic murmur of aortic regurgitation
Panystolic murmur of tricuspid regurgitation
Conti….
2- Myocarditis:
Unexplained congestive heart failure
Tachycardia disproportionate to the fever
Carey Coomb's murmur, a delayed diastolic murmur due to mitral valve involvement
Cardiomegaly
Conduction defects ( low voltage ECG) , arrhythmia and heart blocks
3- Pericarditis:
• Chest pain
• Pericardial friction rub
• Pericardial effusion
• CHF
Chorea (Sydenham's chorea):
It is found in 15% of patients and more common in girls
It is involuntary , purposeless choreiform movement, disappears during sleep and
associated with motor weakness, emotional liability and personality change
May be related to dysfunction basal ganglia and neuronal components
manifest 3--6 months after the onset of acute rheumatic fever
Self-limiting within 2-6 weeks ( up to 6 months)
Erythema Marginatum:
non-pruritic, red macule with demarcated margins.
It manifests over the trunk and the upper arm.
It is an early manifestation of rheumatic fever.
It is not visible in dark skinned patients.
This is often associated with chronic carditis.
Subcutaneous Nodules:
These are late manifestations that occur in 10%-15% of patients
always associated with carditis. (Ninety-five per cent of patients with subcutaneous
nodules will have carditis.
These nodules are small (0.2-2 cm) firm, non-tender , freely mobile and felt over the bony
prominences at the following sites:
• Behind ears
• Occipital region
• Along the spine
• Along the extremities-extensor surfaces of elbow, hands and feet
INVESTIGATIONS:
1- CBC : leukocytosis
2 - ESR : raised , It may be decreased in the following conditions :
(a) Congestive cardiac failure
(b) Mild carditis
(c) Chorea
3- C - reactive protein : increased. If normal rules out acute rheumatic fever
4 – Anti streptolysin 0 (ASO) titer : increased. peaks by 3-6 weeks and remains high for 3-6 months
( other evidence of streptococcal infection : Anti DNase , positive throat culture)
5 - Chest X-ray : cardiomegaly and pulmonary congestion.
6 – ECG : prolongation of P-R interval
7 - ECHO : cardiac dilatation, valve abnormalities and rheumatic vegetations on mitral leaflets
Diagnosis :
Criteria for diagnosis of acute rheumatic fever
In the first episode, two major or one major and two minor criteria plus evidence of
antecedent streptococcal infection are required for the diagnosis of acute rheumatic fever.
Three special categories in which two major or one major and two minor
criteria are not needed form diagnosis are as follows:
1. Chorea : when other causes are ruled out.
2. Indolent carditis : insidious or late onset carditis.
3. Rheumatic fever recurrence
TREATMENT:
1 – Bed rest
2 - Treatment of group A streptococcal infection:
Benzathine penicillin G 1.2 million units (>27 kg) or 0 .6 million units ( <27 kg) intramuscularly single dose after
sensitivity test Or
Procaine penicillin units intramuscularly twice daily for 10 days or
Oral penicillin-V 250 mg 6 hourly for 10 days
For penicillin allergenic patient :
• Azithromycin oral-12.5 mg/kg/day once daily for 5 days
• Cephalexin oral-15-20 mg/kg/dose twice daily for 10 days
3 – Anti inflammatory agents :
(a) Aspirin 100 mg/kg/day for 3-5 days followed by 50 - 75 mg/kg/ day 4-8 weeks .
(b) Steroids : indications are as follows:
• Severe Carditis ( cardiomegaly ,Congestive cardiac failure)
• Aspirin toxicity
• Patients not responding to aspirin
4- Treatment of Congestive Heart Failure :
- cardiac bed - Oxygen
Diuretics ( frusemide +/- spironolactone)
ACE inhibitors ( captopril)
Digoxin ( refractory HF)
5- Treatment of Chorea : ( haloperidol , sodium valproate, carbamazepine , olanzapine)
COMPLICATIONS:
Arrhythmias
Congestive cardiac failure
infective endocarditis
Rheumatic heart disease
Recurrent Rheumatic fever
Sydenham chorea
PREVENTION :
1 - Primary Prevention :
It consists of early recognition and treatment of streptococcal tonsillopharyngitis by appropriate antibiotic therapy.
2 - Secondary Prevention:
The purpose of secondary prophylaxis is to prevent recurrent attacks of rheumatic fever, It means prevention of
colonization of upper respiratory tract in individuals who already have had a previous attack of rheumatic fever or
established RHD.
• Intramuscular benzathine penicillin every 3 weeks
• Macrolides ( azithromycin , erythromycin ) for penicillin allergenic patient
Duration for secondary prevention are as follow:
No carditis : 5 years from the last attack or 21 years of age
Mild – moderate carditis : 10 years from the last attack or 25 years of age
Severe Carditis or RHD : for life
Thanks
