Rheumatic fever
Autoimmune cross-reaction after group A streptococcal pharyngitis (molecular mimicry)
Overview
A multisystem autoimmune sequela of group A streptococcal throat infection, 2–4 weeks later, driven by antibody cross-reactivity. Diagnosed with the Jones criteria. Acute carditis can cause valve damage that, with recurrences, leads to chronic rheumatic heart disease (especially mitral stenosis) — still a major cause of valve disease worldwide. Long-term penicillin prophylaxis prevents recurrence.
Recognise
- 2–4 weeks after a sore throat: migratory polyarthritis, carditis (new murmur/pericarditis), Sydenham chorea, erythema marginatum, subcutaneous nodules
- Jones criteria: major (carditis, polyarthritis, chorea, erythema marginatum, subcutaneous nodules) + minor (fever, arthralgia, raised ESR/CRP, prolonged PR) with evidence of preceding strep infection
- Chronic rheumatic heart disease → mitral stenosis (commonest), then aortic and other valves
Red flags
- Severe carditis with heart failure → admit and treat
- Sydenham chorea may appear late/in isolation — still indicates rheumatic fever
Differentials & how to tell them apart
Investigations
Evidence of preceding GAS infection (throat swab, rapid antigen, anti-streptolysin O/anti-DNase B titres); inflammatory markers; ECG (prolonged PR); echo (carditis/valve involvement); apply the Jones criteria.
Management
Penicillin to treat GAS + anti-inflammatory therapy + long-term penicillin prophylaxis
- 1Diagnose with the Jones criteria plus evidence of preceding GAS infection. Treat the streptococcal infection with penicillin and control inflammation/arthritis with aspirin/NSAIDs.Gate: Carditis with heart failure → admit and treat; the presence/severity of carditis determines how long prophylaxis continues.
- 2Start long-term penicillin prophylaxis (IM benzathine penicillin) to prevent recurrent attacks and progression to chronic rheumatic heart disease; echo surveillance of valves.
Key points
Migratory polyarthritis + carditis + chorea + erythema marginatum 2–4 weeks after a sore throat = rheumatic fever (Jones criteria + strep evidence). It causes chronic mitral stenosis. Treat the strep, give anti-inflammatories, and start long-term penicillin PROPHYLAXIS to prevent recurrence.
Monitor & prognosis
Echo for valve damage, recurrence surveillance, prophylaxis adherence.
Recurrences worsen valve damage; prophylaxis is key — chronic rheumatic heart disease is the long-term sequela.
Source: WHO/Jones criteria; cross-ref child_health (Sydenham chorea)