Acute rheumatic fever

Background

• Post-infectious complication of Streptococcus pyogenes infection
• Caused by certain serotypes of M protein

Clinical Manifestation

• Presents 1 to 5 weeks following a streptococcal infection
• Clinical syndrome characterized by fever, carditis, polyarticular arthritis, subcutaneous nodules, Sydenham chorea, and erythema marginatum
  • Diagnosed using the Modified Jones criteria
• Patients with a history of acute rheumatic fever or rheumatic heart disease may have recurrences with subsequent reinfection with Streptococcus pyogenes
  • Recurrences do not require as many criteria to diagnose
  • Still need to exclude other possibilities

Differential Diagnosis

Arthritis

• Septic arthritis including gonococcal arthritis
• Connective tissue disease or other autoimmune diseases include juvenile idiopathic arthritis
• Viral arthropathy
• Reactive arthropathy
• Lyme disease
• Sickle cell anemia
• Infective endocarditis
• Leukemia or lymphoma
• Gout or pseudogout
• Post-streptococcal reactive arthritis
• Henoch-Schonlein purpura

Carditis

• Physiological mitral valve regurgitation
• Mitral valve prolapse
• Myxomatous mitral valve
• Fibroelastoma
• Congenital mitral valve disease
• Congenital aortic valve disease
• Infective endocarditis
• Cardiomyopathy
• Myocarditis (including viral and idiopathic)
• Kawasaki disease

Chorea

• Drug intoxication
• Wilson disease
• Tic disorder
• Choreoathetoid cerebral palsy
• Encephalitis
• Familial chorea, including Huntington disease
• Intracranial tumour
• Lyme disease
• Hormonal
• Metabolic, including Lesch-Nyhan, hyperalaninemia, ataxia-telangiectasia
• Antiphospholipid antibody syndrome
• Systemic lupus erythematosus
• Vasculitis
• Sarcoidosis
• Hyperthyroidism

Diagnosis

• Acute rheumatic fever is diagnosed using the modified Jones criteria

Management

Acute management

• Supportive care
  • Arthritis: NSAID, such as aspirin, ibuprofen, or naproxen, until all joint symptoms are resolved
  • Fever: acetaminophen, if desired
  • Heart failure: diuretics if mild or moderate; add ACE inhibitor if severe
  • Atrial fibrillation: digoxin
  • Chorea: if moderate, can use carbamazepine or valproate; if severe, can add risperidone, haloperidol, or diazepam; and if persistent or very severe, can add prednisone or methylprednisolone
• Decolonization, achieved by treating as streptococcal pharyngitis with either benzathine penicillin G 1.2 MU IM once or amoxicillin 500 mg PO bid for 10 days

Secondary prevention

• Needed to prevent further infections with Streptococcus pyogenes, which most commonly manifests as pharyngitis or pyoderma
• Choice of antibiotic:
  • The best evidence is benzathine penicillin G 1.2 MU every 2 to 4 weeks (for adults and children ≥20 kg) or 600 KU (for children <20 kg)
    • Although every 2 weeks provides the best protection, every 3 to 4 weeks is still good (and more common, since it is more acceptable to patients)
    • Can, for example, suggest patients get their injection every full moon
  • Alternatively, can use oral penicillin V 250 mg PO bid
  • If allergy, can use any alternative antibiotic, though they are inferior to penicillin
• Duration, per Australian guidelines:
  • Possible: 12 months, then reassess
  • Probable: for at least 5 years and until at least 21 years of age
  • Definite, without carditis: for at least 5 years and until at least 21 years of age
  • Definite, with carditis:
    • Mild RHD: for at least 10 years (or 5 years if no preceding ARF) and until at least 21 years of age
    • Moderate RHD: for at least 10 years (or 5 years if no preceding ARF) and until at least 35 years of age
    • Severe RHD: for at least 10 years (or 5 years if no preceding ARF) and until at least 40 years of age

Further Reading

• The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020. Available online.
• Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography. Circulation. 2015;131:1806-1818. doi: 10.1161/CIR.0000000000000205