Acute rheumatic fever

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  • Primarily affects school age children 2-6wk after strep pharyngitis
  • Connective tissue of heart, joints, CNS, subq tissues are targeted by immune reaction

Clinical Features

  • Polyarthritis
    • Most common symptom (75%)
    • Migratory, fleeting polyarticular arthritis primarily affecting large joints
  • Carditis (33%)
    • Most serious complication and second most common
      • Tachycardia out of proportion to the degree of fever is common; its absence makes the diagnosis of myocarditis unlikely.[1]
      • New murmur, pericardial rub, CHF
  • Chorea (10%)
    • May appear months following strep infection, may be sole manifestation of RF
  • Erythema marginatum
    • Persists only for several days
    • Usually coexists with presence of carditis in some form
    • Nonpruritic, located on trunk and proximal limbs, never on face
  • Nodules
    • Located on extensor surfaces of wrists, elbows, knees

Differential Diagnosis

  • Kawasaki Disease
  • Viral or other forms of cardiomyopathy
  • Leukemia
  • Vasculitis (HSP, drug reaction)

Pediatric hip pain


Algorithm for Polyarticular arthralgia



Modified Jones Criteria (1992) for Acute Rheumatic Fever

REQUIRE: 2 major or 1 major and 2 minor criteria and evidence of previous GAS pharyngitis

  • Major diagnostic criteria
    • Carditis (new or changing murmur, cardiomegaly, CHF, pericarditis)
    • Migratory polyarthritis (typically affects knees, ankles, elbows, wrists)
    • Chorea (abrupt, purposeless movements)
    • Subcutaneous nodules (painless, firm, usually over bones/tendons)
    • Erythema marginatum (non-pruritic, trunk/extremities, with facial sparing)
  • Minor diagnostic criteria
    • Fever
    • Arthralgia
    • History of previous attack of rheumatic fever
    • Prolonged PR interval
    • Elevated ESR, CRP
  • Evidence of preceding streptococcal infection
    • Increased ASO or other strep ab
    • Positive throat culture for Group A strep
    • Positive rapid GAS
    • Recent scarlet fever


  1. Penicillin
    • Indicated for all with rheumatic fever even if culture for strep is negative
    • 600K units IM if <27 kg
    • 1.2 million units IM if >27 kg
    • Penicillin V PO x10d
    • Erythromycin x10d if pen allergic
    • Prophylaxis
Category Duration
Rheumatic fever without carditis 5 years or until age 21 years, whichever is longer
Rheumatic fever with carditis but without residual heart disease (no valvular disease) 10 years or well into adulthood, whichever is longer
Rheumatic fever with carditis and residual heart disease (persistent valvular disease) 10 years since last episode and at least until age 40 years; most commonly lifelong
  1. Arthritis
    • High-dose aspirin therapy (75-100mg/kg/d)
  2. Carditis
  3. Chorea


  • Admit for confirmation of diagnosis

See Also


  1. Park M: Parks's Pediatric Cardiology for Practitioners, ed 6. Philadelphia, Saunders-Elsevier., 2014, (Ch) 20: p 368.
  • Horeczko T, Inaba AS: Cardiac Disorders; in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 8. St. Louis, Mosby, Inc., 2014, (Ch) 171: p 2139-2170.
  • Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Associa