Acute rheumatic fever: Clinical manifestations and diagnosis
- Allan Gibofsky, MD, JD, FACP, FCLM
Allan Gibofsky, MD, JD, FACP, FCLM
- Professor of Medicine and Public Health
- Weill Medical College of Cornell University
- Section Editors
- Robert Sundel, MD
Robert Sundel, MD
- Section Editor — Pediatric Rheumatology
- Associate Professor of Pediatrics
- Harvard Medical School
- Daniel J Sexton, MD
Daniel J Sexton, MD
- Editor-in-Chief — Infectious Diseases
- Section Editor — Bacterial Infections
- Professor of Medicine
- Duke University Medical Center
Acute rheumatic fever (ARF) is a nonsuppurative sequela that occurs two to four weeks following group A Streptococcus pharyngitis and may consist of arthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules. Damage to cardiac valves may be chronic and progressive, resulting in cardiac decompensation.
The clinical manifestations and diagnosis of ARF are reviewed here. The epidemiology, pathogenesis, treatment, and prevention of this disorder are presented separately. (See "Acute rheumatic fever: Epidemiology and pathogenesis" and "Acute rheumatic fever: Treatment and prevention".)
Acute illness — ARF can present with several different clinical findings within weeks of a group A streptococcal (GAS) tonsillopharyngitis (or streptococcal pyoderma in patients from tropical regions) [1-3]. The possible major and minor manifestations are reviewed here (table 1). The criteria for diagnosis according to the modified Jones criteria  are reviewed below. (See 'Diagnosis' below.)
The five major manifestations (and percent of patients with each) are :
●Carditis and valvulitis (eg, pancarditis) that is clinical or subclinical – 50 to 70 percent
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- CLINICAL MANIFESTATIONS
- Acute illness
- - Arthritis
- - Carditis
- - Sydenham chorea
- - Erythema marginatum
- - Subcutaneous nodules
- - Additional features
- Late sequelae
- - Rheumatic heart disease
- - Jaccoud arthropathy
- Diagnostic criteria
- - Exceptions
- - Moderate/high-risk populations
- Confirmation of group A streptococcal (GAS) infection
- Laboratory studies
- Cardiac evaluation
- DIFFERENTIAL DIAGNOSIS
- Poststreptococcal reactive arthritis