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Rheumatic fever
Background
Overview
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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Guidelines
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 European Society for Microbiology and Infectious Diseases (ESCMID 2012), the Infectious Diseases Society of America (IDSA 2012), and the American Heart Association (AHA/AAP 2009).
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Screening and diagnosis
Diagnostic criteria: as per AHA 2015 guidelines, 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.
C
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Revised Jones criteria for acute rheumatic fever
Population risk
Low risk
Moderate or high risk
History of acute rheumatic fever or rheumatic heart disease
Yes
No
Evidence of preceding group A Streptococcus infection
Increased or rising anti-streptolysin O titer or other streptococcal antibodies (anti-DNase B)
Positive rapid group A streptococcal carbohydrate antigen test in a pediatric patient with clinical presentation suggesting a high pretest probability of streptococcal pharyngitis
Positive throat culture for group A β-hemolytic streptococci
Major criteria
Clinical or subclinical carditis (subclinical carditis indicates echocardiographic valvulitis with findings such as pathologic MR or aortic regurgitation, or morphologic valvulitis)
Arthritis (monoarthritis or polyarthritis in low-risk populations, polyarthritis-only in moderate-to-high risk populations)
Chorea
Erythema marginatum
Subcutaneous nodules
Minor criteria
Prolonged PR interval (after accounting for age variability)
Arthralgia (polyarthralgia in low-risk populations, monoarthralgia in high-risk populations)
Fever (≥ 38.5 °C or 101.3 °F in low-risk populations, ≥ 38.0 °C or 100.4 °F in moderate-to-high-risk populations)
Increased ESR (≥ 60 mm in the first hour in low-risk populations, ≥ 30 mm/hour in moderate-to-high-risk populations) and/or CRP (≥ 3.0 mg/dL)
Diagnostic criteria for ARF are not met
Classification and risk stratification
Diagnostic investigations
Laboratory tests: as per AHA 2015 guidelines, 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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Echocardiography
Specific circumstances
Patients with post-streptococcal reactive arthritis: as per AAP/AHA 2009 guidelines, 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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Preventative measures
Primary prevention, testing: as per IDSA 2012 guidelines, 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