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An autoimmune, inflammatory complication of untreated or inadequately treated Group A Streptococcus (GAS) pharyngitis, affecting joints, heart, skin, and CNS.
An autoimmune, inflammatory complication of untreated or inadequately treated Group A Streptococcus (GAS) pharyngitis, affecting joints, heart, skin, and CNS.
Risk Factors
Recent Group A Strep pharyngitis (usually 2–4 weeks prior)
Age 5–15 years
Low socioeconomic status, overcrowding, poor access to healthcare
Family history of rheumatic fever or RHD
Indigenous Australian and Pacific Islander populations at higher risk
Aetiology
Immune-mediated cross-reactivity between GAS antigens and human tissues
Molecular mimicry – antibodies against streptococcal M protein cross-react with heart, joints, brain, and skin
Follows untreated or partially treated GAS throat infection (not skin infection)
Pathophysiology
Inflammatory lesions in connective tissue, especially heart (pancarditis – endo, myo, peri)
Valvular damage, especially mitral (most common) and aortic valves
Acute inflammation may subside, but valve damage may be permanent, leading to chronic RHD
Recurrent episodes cause cumulative damage
Diagnosis
Jones Criteria (2 major or 1 major + 2 minor + evidence of preceding GAS infection)
Major criteria:
Migratory polyarthritis (large joints, asymmetric)
Carditis (pancarditis – murmur, tachycardia, pericardial rub, CCF)
Sydenham’s chorea (involuntary movements)
Erythema marginatum (pink, serpiginous rash on trunk/limbs)
Subcutaneous nodules (painless, firm, over extensor surfaces)
Minor criteria:
Fever, arthralgia
↑ ESR or CRP
Prolonged PR interval on ECG
Previous rheumatic fever
Supporting evidence of GAS infection:
Positive throat culture or rapid antigen test
Elevated/rising ASO or anti-DNase B titres
Differential Diagnosis
Juvenile idiopathic arthritis
Infective endocarditis
SLE
Post-streptococcal reactive arthritis
Viral myocarditis
Investigations
Throat swab for GAS
ASO titre, anti-DNase B
FBC – leukocytosis
CRP, ESR – elevated
ECG – PR prolongation, arrhythmia
Echo – valve regurgitation, pericardial effusion, chamber dilation
Management
Antibiotics – benzathine penicillin IM stat, or oral penicillin for 10 days
Aspirin or NSAIDs – for arthritis and fever
Prednisolone – for severe carditis
Haloperidol or sodium valproate – for Sydenham’s chorea
Heart failure management if needed
Bed rest in acute phase
Secondary prophylaxis
Benzathine penicillin IM every 21–28 days
Duration of prophylaxis
5 years or until age 21 (whichever is longer) for 1st episode with no carditis
10 years or until age 35 for carditis with no residual disease
≥10 years or lifelong for carditis with residual valve disease
Complications
Acute
Heart failure
Pericardial effusion
Chorea affecting school performance
Hospitalisation
Chronic
Rheumatic heart disease (valvular regurgitation/stenosis, especially mitral)
Recurrent episodes worsen valve damage
Atrial fibrillation
Infective endocarditis risk
Prognosis
Good with early detection and secondary prophylaxis
Worse with recurrent episodes or delayed treatment
Leading cause of acquired heart disease in children in endemic areas
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