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Rheumatic fever

Definition
RF is a multiorgan autoimmune disease caused by group A β-hemolytic streptococcal infection in individuals with a genetic predisposition.
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Pathophysiology
RF is predominantly caused by S. pyogenes or group A β-hemolytic Streptococcus.
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Epidemiology
The incidence of RF varies from < 0.5 to > 100 per 100,000 person-years in highly developed and developing countries, respectively.
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Disease course
Group A streptococcus infection in susceptible individuals produces antigens that activate specific B and T cells through molecular mimicry triggering autoimmune reactions against host tissues (heart, brain, joints, skin) resulting in rheumatic carditis, rheumatic heart disease and its complications (AF, endocarditis, embolic stroke, HF), chorea arthritis, and erythema marginatum and subcutaneous nodules.
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Prognosis and risk of recurrence
The cumulative incidence of progression to rheumatic heart disease at 1 year, 5 years, and 10 years is 27.1%, 44.0%, and 51.9%, respectively. The cumulative incidence of RF recurrence at 10 years is 19.8%.
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Key sources
The following summarized guidelines for the evaluation and management of rheumatic fever are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ACC 2021), the American Heart Association (AHA 2015), the Infectious Diseases Society of America (IDSA 2012), the European Society for Microbiology and Infectious Diseases (ESCMID 2012), and the American Heart Association (AHA/AAP 2009).
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Guidelines

1.Screening and diagnosis

Diagnostic criteria: consider making a presumptive diagnosis of ARF based on 2 major or 1 major and 2 minor or 3 minor manifestations in patients with a reliable past history of ARF or established rheumatic heart disease and in the face of documented group A streptococcal infection.
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2.Classification and risk stratification

Risk stratification: as per AHA 2015 guidelines, consider stratifying persons being at low risk for ARF if they come from a setting or population known to experience low rates of ARF or rheumatic heart disease.
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3.Diagnostic investigations

Laboratory tests: view any of the following as evidence of preceding infection:
increased or rising anti-streptolysin O titer or other streptococcal antibodies (anti-DNAse B)
positive throat culture for group A β-hemolytic streptococci
positive rapid group A streptococcal carbohydrate antigen test in a pediatric patient with clinical presentation suggesting a high pretest probability of streptococcal pharyngitis
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4.Specific circumstances

Patients with post-streptococcal reactive arthritis: recognize that no more than half of patients with post-streptococcal reactive arthritis patients who have a throat culture performed have group A Streptococcus infection isolated, even though all patients have serological evidence of recent group A Streptococcus infection.
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5.Preventative measures

Primary prevention, testing, IDSA: obtain a rapid antigen detection test for GAS pharyngitis in most patients with symptoms of acute pharyngitis because the clinical features alone do not reliably discriminate between group A streptococcal and viral pharyngitis, except when overt viral features such as rhinorrhea, cough, oral ulcers, and/or hoarseness are present.
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  • Primary prevention (antibiotic therapy)

  • Primary prevention (chronic GAS carriers)

  • Secondary prevention