Post-streptococcal autoimmune sequela causing pancarditis acutely and chronic valvular damage (mitral > aortic) years later.
Also known as: ARF, acute rheumatic fever, RHD, rheumatic heart disease, Jones criteria
Overview
Acute rheumatic fever (ARF) is a delayed, immune-mediated inflammatory disease following untreated group A streptococcal (GAS) pharyngitis, characterized by carditis, arthritis, chorea, and skin findings. Rheumatic heart disease (RHD) refers to the chronic valvular sequelae, classically progressive mitral stenosis, that develop years to decades after one or more episodes of ARF.
Epidemiology
Rare in industrialized nations but a leading cause of cardiovascular morbidity in low-resource regions; >40 million people live with RHD worldwide. Peak incidence of ARF is 5-15 years. Mitral valve is involved in roughly 70% of RHD cases; combined mitral and aortic involvement in ~25%.
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Age 5-15 years for primary attack; recurrences may extend into adulthood
Pathophysiology
Molecular mimicry between GAS M-protein and human cardiac myosin, valve glycoproteins, and neuronal antigens triggers cross-reactive antibody and T-cell responses. Acutely, this produces pancarditis (endocarditis, myocarditis, pericarditis), arthritis, and CNS inflammation. Chronic, repeated bouts of valvulitis cause leaflet thickening, commissural fusion, and chordal shortening, producing the characteristic fish-mouth deformity of rheumatic mitral stenosis.
Clinical presentation
Symptoms
Migratory polyarthritis of large joints (knees, ankles, elbows, wrists) — exquisitely responsive to salicylates
Carditis: new murmur, chest discomfort, dyspnea, fatigue, palpitations
Sydenham chorea: involuntary purposeless movements, emotional lability, milkmaid's grip — appears weeks to months after pharyngitis, may be the sole manifestation
Fever, malaise, anorexia following pharyngitis 2-4 weeks earlier
In chronic RHD: progressive exertional dyspnea, orthopnea, palpitations from AFib, hemoptysis
Signs / physical exam
New apical pansystolic murmur (mitral regurgitation), Carey Coombs mid-diastolic murmur, or early diastolic murmur of AR
Erythema marginatum: evanescent, serpiginous, non-pruritic pink rings on trunk
Subcutaneous nodules: firm, painless, over extensor surfaces (rare, severe disease)
Pericardial friction rub or effusion in carditis
Chronic RHD: opening snap and rumbling mid-diastolic murmur of MS, irregularly irregular pulse (AFib)
Classic findings
Migratory polyarthritis + new murmur + recent pharyngitis = ARF until proven otherwise. Erythema marginatum and subcutaneous nodules are highly specific but uncommon.
Differential diagnosis
Reactive arthritis (post-streptococcal arthritis without other Jones criteria) — Persistent migratory or additive arthritis with minimal response to NSAIDs; no carditis or chorea; ASO titer elevated but lacks Jones threshold
Juvenile idiopathic arthritis (JIA) — Insidious onset, morning stiffness, may have uveitis; arthritis pattern is symmetric and chronic, not migratory; negative ASO
Viral myocarditis — Recent viral prodrome, no migratory arthritis or chorea; elevated troponin and global LV dysfunction
Diagnostic workup
Diagnostic criteria
2015 Revised Jones Criteria (AHA): initial ARF requires 2 major OR 1 major + 2 minor, with evidence of preceding GAS infection. Recurrent ARF allows 3 minor in addition. MAJOR (low-risk populations): carditis (clinical or subclinical), polyarthritis, chorea, erythema marginatum, subcutaneous nodules. MINOR: polyarthralgia, fever ≥38.5°C, ESR ≥60 or CRP ≥3, prolonged PR. Moderate/high-risk populations have less stringent major criteria (monoarthritis or polyarthralgia can count, fever threshold ≥38°C).
Labs
Throat culture or rapid antigen detection (often negative by time of ARF presentation)
Anti-streptolysin O (ASO) titer — rises 1 week, peaks 3-6 weeks; combine with anti-DNase B for sensitivity >95%
ESR and CRP (elevated; used as minor criteria)
CBC (mild leukocytosis), BMP
Blood cultures to exclude infective endocarditis
Imaging
Transthoracic echocardiography in all suspected cases — detects subclinical carditis (mitral regurgitation jet, leaflet thickening); now incorporated into 2015 revised Jones criteria
12-lead ECG: prolonged PR interval (minor criterion), AV block, AFib in chronic RHD
CXR: cardiomegaly, pulmonary congestion if HF present
Diagnostic algorithm
Manifestation
Category
Notes
Carditis (clinical or subclinical)
Major
Most consequential — drives long-term morbidity
Polyarthritis (or monoarthritis in high-risk)
Major
Migratory, large joints, exquisitely aspirin-responsive
Sydenham chorea
Major
Latent period months; may be sole feature
Erythema marginatum
Major
Evanescent, non-pruritic, trunk and proximal limbs
Subcutaneous nodules
Major
Painless, extensor surfaces, severe disease
Polyarthralgia (low-risk) / Fever ≥38.5°C
Minor
Counts only if not used as major
ESR ≥60 or CRP ≥3.0 mg/dL
Minor
Acute-phase reactants
Prolonged PR interval
Minor
Not counted if carditis is major
2015 Revised Jones Criteria for diagnosis of initial acute rheumatic fever (low-risk populations).
Treatment
First-line
Eradicate streptococcal infection: penicillin G benzathine 1.2 million units IM once (or 600,000 units if <27 kg); alternative oral penicillin V or amoxicillin × 10 days; azithromycin or cephalexin if penicillin allergic
Anti-inflammatory for arthritis and mild carditis: high-dose aspirin 50-100 mg/kg/day divided QID (taper over weeks based on symptoms/inflammatory markers); naproxen is increasingly used as an alternative
Severe carditis with heart failure: corticosteroids (prednisone 1-2 mg/kg/day, taper over 2-4 weeks) plus standard HF therapy
Bed rest until inflammation subsides; gradual return to activity
Secondary prophylaxis (essential after ARF)
Penicillin G benzathine 1.2 million units IM every 4 weeks (every 3 weeks in high-risk regions)
Alternatives: oral penicillin V 250 mg BID, sulfadiazine, or macrolide if allergic
Duration: ARF without carditis — 5 years or until age 21 (whichever longer); ARF with carditis, no residual disease — 10 years or until age 21; carditis with residual valvular disease — 10 years or until age 40 (lifelong if recurrent or severe)
Second-line / adjunct
Sydenham chorea: usually self-limited; for disabling symptoms use carbamazepine or valproic acid; haloperidol historically used but more side effects
Valve surgery (balloon valvotomy, repair, or replacement) for severe symptomatic RHD
Complications
Chronic valvular disease, especially rheumatic mitral stenosis (most common chronic sequel)
Atrial fibrillation with thromboembolic events (stroke, systemic emboli)
Heart failure, pulmonary hypertension
Infective endocarditis superimposed on damaged valves
Recurrent ARF without adequate secondary prophylaxis
PANCE pearls
Jones criteria require evidence of preceding GAS infection EXCEPT in chorea or indolent carditis, where the latent interval can be months.
Throat culture is often negative by the time ARF develops; rely on ASO and anti-DNase B titers.
Aspirin classically rapidly resolves arthritis within 48 hours; failure to respond should prompt reconsideration of the diagnosis.
Subclinical carditis (echo-detected MR without auscultatory findings) now counts as a major criterion in the 2015 update.
Rheumatic mitral stenosis is the prototypical cause of MS worldwide and characteristically has an opening snap with mid-diastolic rumble and presystolic accentuation if in sinus rhythm.
References
AHA 2015 — Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography (Gewitz et al., Circulation 2015)
AHA/ACC 2020 — 2020 ACC/AHA Guideline for the Management of Patients with Valvular Heart Disease (Otto et al., Circulation 2021)
WHF 2012 — World Heart Federation Criteria for Echocardiographic Diagnosis of RHD (Reményi et al., Nat Rev Cardiol 2012)
IDSA 2012 — IDSA Clinical Practice Guideline for the Diagnosis and Management of Group A Streptococcal Pharyngitis (Shulman et al., CID 2012)
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