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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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