What is rheumatic fever, particularly in relation to a child or adolescent recently diagnosed with streptococcal pharyngitis (strep throat)?

What is Rheumatic Fever?

Rheumatic fever is a delayed autoimmune inflammatory disease that develops 2-3 weeks after untreated or inadequately treated Group A streptococcal (GAS) pharyngitis, capable of causing permanent cardiac valve damage and lifelong disability. 1, 2, 3

Pathophysiology and Timing

• Rheumatic fever occurs as a non-suppurative complication following a symptom-free interval of 14-21 days after the initial streptococcal throat infection 2, 3
• The disease represents an autoimmune phenomenon triggered by molecular mimicry between streptococcal antigens and human tissue 3, 4
• Critically, at least one-third of rheumatic fever cases result from inapparent (asymptomatic) streptococcal infections, meaning the child may never have had recognized symptoms of strep throat 1, 2

Clinical Manifestations

The diagnosis requires meeting Jones criteria: two major criteria OR one major plus two minor criteria, plus evidence of preceding GAS infection 2. The major criteria include:

• Carditis (inflammation of the heart, potentially causing permanent valve damage) 2, 3
• Polyarthritis (migratory joint inflammation affecting multiple joints) 3, 4
• Chorea (involuntary movements, also called Sydenham chorea) 1, 3
• Subcutaneous nodules 3, 4
• Erythema marginatum (characteristic rash) 3, 4

Why This Matters for Your Child

• Rheumatic fever carries significant risk of permanent cardiac damage, with potential for severe rheumatic heart disease requiring lifelong medical management 2
• The disease remains a leading cause of cardiovascular death in the first five decades of life in affected populations 3
• Recurrent attacks can worsen existing heart damage or cause new cardiac disease, even in children who didn't develop heart problems during the first attack 1

Prevention Through Treatment of Strep Throat

• Appropriate antibiotic treatment of streptococcal pharyngitis prevents acute rheumatic fever in most cases 1
• Treatment must be initiated within 9 days of symptom onset to prevent rheumatic fever 5
• The recommended treatment is penicillin V (250 mg 2-3 times daily for children <60 lb; 500 mg 2-3 times daily for children ≥60 lb and adolescents) for 10 days 1
• For penicillin-allergic patients, alternatives include first-generation cephalosporins, azithromycin (5 days), or clindamycin (10 days) 1

Critical Clinical Pitfall

The most important caveat is that rheumatic fever is not always preventable—even with optimal treatment of symptomatic strep throat, recurrences can occur, and many cases arise from infections that were never symptomatic enough to prompt medical attention 1, 2. This is why children who develop rheumatic fever require continuous antimicrobial prophylaxis rather than just treating individual infections 1.

If Rheumatic Fever Develops

• A full therapeutic course of penicillin must be given immediately to eradicate residual GAS, even if throat culture is negative 1
• Continuous antimicrobial prophylaxis is mandatory to prevent recurrent attacks 1, 2
• Duration of prophylaxis depends on severity: 5-10 years or until age 21-40 years, sometimes lifelong for those with persistent valve disease 1

Epidemiology Relevant to Your Situation

• Rheumatic fever primarily affects children aged 5-15 years 1
• It is rare in children younger than 3 years of age 1
• In temperate climates, GAS pharyngitis typically occurs in winter and early spring 1
• The disease affects nearly 20 million people globally and remains a major cause of morbidity in developing regions and disadvantaged populations 3, 6