Rheumatic fever

Rheumatic fever (also known as acute rheumatic fever, ARF) is an inflammatory disease that occurs as a delayed complication of untreated or inadequately treated group A streptococcal (GAS) pharyngitis (strep throat). It is classified as a post-infectious autoimmune disorder in which the body's immune response to the streptococcal infection cross-reacts with the patient's own tissues, particularly affecting the heart, joints, skin, and central nervous system. Rheumatic fever primarily affects children and adolescents between the ages of 5 and 15 years, though it can occur in older individuals. The hallmark clinical features are defined by the revised Jones criteria and include carditis (inflammation of the heart, which can affect the valves, myocardium, and pericardium), migratory polyarthritis (joint inflammation that moves from one large joint to another), Sydenham chorea (involuntary movements caused by central nervous system involvement), erythema marginatum (a distinctive non-pruritic rash with pink rings on the trunk and limbs), and subcutaneous nodules (firm, painless nodules found over bony prominences). Carditis is the most serious manifestation, as it can lead to permanent rheumatic heart disease (RHD), particularly mitral and aortic valve damage, which remains a leading cause of acquired heart disease in children and young adults worldwide. Treatment involves eradication of the streptococcal infection with antibiotics (typically penicillin), anti-inflammatory therapy with aspirin or corticosteroids for carditis and arthritis, and supportive care for chorea. Long-term secondary prophylaxis with regular penicillin injections is essential to prevent recurrent streptococcal infections and subsequent episodes of rheumatic fever, which increase the risk of progressive heart valve damage. Severe rheumatic heart disease may ultimately require surgical valve repair or replacement. The disease is now rare in high-income countries due to improved living conditions and access to antibiotics but remains a significant public health burden in low- and middle-income countries, particularly in sub-Saharan Africa, South Asia, and among Indigenous populations in Australia and New Zealand.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Rheumatic fever