Cardiovascular
AKT · Cardiovascular/Valves & endocardiumlow yield

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

Septic/reactive arthritissingle hot joint with positive cultures; not migratory
Juvenile idiopathic arthritis / SLEchronic course, autoantibodies
Infective endocarditispositive blood cultures, vegetations
Other chorea (Huntington, drug-induced)no strep evidence, different context

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

  1. 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.
  2. 2Start long-term penicillin prophylaxis (IM benzathine penicillin) to prevent recurrent attacks and progression to chronic rheumatic heart disease; echo surveillance of valves.
Benzylpenicillin/phenoxymethylpenicillin to eradicate GAStreat the streptococcal infection
NSAIDs (aspirin) for arthritis/carditisanti-inflammatory symptom control
Long-term penicillin prophylaxis (IM benzathine penicillin)prevents recurrence — continued for years/until adulthood depending on carditis
Treat heart failure/choreasupportive; corticosteroids for severe carditis in some cases

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)