Studies in the 1950s during an epidemic on a military base showed a 3% incidence of rheumatic fever in adults with streptococcal pharyngitis left untreated.
Pathophysiology
Rheumatic fever follows GABHS (Streptococcus pyogenes) pharyngitis. The organisms attach to upper respiratory epithelial cells and produce enzymes that let them damage and invade tissue. After a 2-4 day incubation, they provoke an acute inflammatory response with 3-5 days of sore throat, fever, malaise, headache, and elevated leukocyte count. In a small percentage, infection leads to rheumatic fever several weeks after the sore throat resolves; only pharyngeal infections initiate or reactivate it. Oral and respiratory secretions transmit the organism, crowding enhances spread, and patients stay infectious for weeks after symptoms clear. Severe valve scarring develops over months to years after an acute episode, and recurrences cause progressive damage. The mitral valve is affected most commonly and severely (65-70% of patients); the aortic valve is second (25%).
Statistics and Incidences
Rheumatic fever is most common in 5- to 15-year-old children, though it occurs in younger children and adults. US RHD prevalence was less than 0.05 per 1000 population, with rare regional outbreaks in Tennessee in the 1960s and in Utah, Ohio, and Pennsylvania in the 1980s. A review of US acute rheumatic fever admissions from 2001-2011 showed steady increases since 2001, peaking in 2005, then declining. Worldwide, the burden is far larger than older estimates suggested: the Global Burden of Disease study counted about 33.4 million prevalent cases of RHD and roughly 319,400 deaths in 2015, and prevalence has continued to climb since (Watkins et al., NEJM 2017; GBD 2019, JAHA). RHD is the major cause of morbidity from rheumatic fever and the leading cause of mitral insufficiency and stenosis in the United States and worldwide. Native Hawaiians and Maori (both of Polynesian descent) carry a higher incidence, 13.4 per 100,000 hospitalized children per year even with antibiotic prophylaxis. Males and females are affected in equal numbers, but females fare worse and have a slightly higher incidence of chorea. The median age is 10 years; GABHS pharyngitis is uncommon under age 3 years, and acute rheumatic fever is extremely rare in those younger children in industrialized countries.
Causes
The disease is an autoimmune response, though the exact pathogenesis stays unclear. It develops only after GABHS pharyngitis, and only pharyngeal infections initiate or reactivate it. Molecular mimicry between streptococcal and human proteins involves both B and T cells, with T-cell infiltration of the heart; increased inflammatory cytokine production is thought to be the final step that damages cardiac tissue in RHD. Streptococcal antigens structurally similar to cardiac tissue include hyaluronate in the bacterial capsule, cell wall polysaccharides (similar to heart valve glycoproteins), and membrane antigens sharing epitopes with sarcolemma and smooth muscle. Decreased regulatory T-cell levels are associated with RHD and greater severity.
Clinical Manifestations
The modified Jones criteria (revised in 1992 and again in 2016) guide diagnosis. For recurrent rheumatic fever they require 2 major, or 1 major and 2 minor, or 3 minor criteria.
Major Diagnostic Criteria
Carditis (clinical and/or subclinical on echo); polyarthritis (monoarthritis or polyarthralgia is adequate in moderate/high-risk populations, with polyarthralgia requiring exclusion of more likely causes); chorea (jerky, uncontrollable movements, Sydenham chorea or St. Vitus' dance, most often in hands, feet, and face); subcutaneous nodules (small, painless bumps beneath the skin); and erythema marginatum (flat or slightly raised, painless rash with a ragged edge).
Minor Diagnostic Criteria
Fever of ≥38.5°C (≥38°C in moderate/high-risk populations); polyarthralgia (painful, tender joints, most often knees, ankles, elbows, and wrists); prolonged PR interval for age on ECG; and elevated peak ESR during acute illness ≥60 mm/h and/or C-reactive protein ≥3.0 mg/dl.
Assessment and Diagnostic Findings
There is no single test; diagnosis rests on history, exam, and test results. Throat cultures for GABHS are usually negative by the time rheumatic fever or RHD appears, so try to isolate the organism before starting antibiotics to confirm streptococcal pharyngitis and allow typing. The rapid antigen detection test identifies group A streptococci antigen, letting you diagnose and start antibiotics in the office. Antistreptococcal antibody levels peak as rheumatic fever begins, so they confirm prior GAS infection and are especially useful when chorea is the only criterion. Acute-phase reactants (C-reactive protein and ESR) are elevated, with high sensitivity but low specificity. Heart-reactive antibodies: tropomyosin is elevated in acute rheumatic fever. The rapid D8/17 test, an immunofluorescence technique for the B-cell marker D8/17, is positive in 90% of patients and may flag those at risk. Chest radiography may show cardiomegaly, pulmonary congestion, and other heart failure findings. Echocardiography identifies and quantifies valve insufficiency and ventricular dysfunction in acute RHD.
Medical Management
Therapy targets the GABHS pharyngitis (if still present), suppresses the autoimmune inflammation, and supports any congestive heart failure (CHF). Anti-inflammatory treatment uses salicylates and steroids; aspirin in anti-inflammatory doses reduces every manifestation except chorea, usually dramatically. For moderate to severe carditis (cardiomegaly, third-degree heart block, or CHF), add PO prednisone to salicylate therapy. For severe Sydenham chorea movements, an anticonvulsant such as valproic acid (Depakene) or carbamazepine (Carbatrol, Tegretol) may be prescribed. Penicillin or another antibiotic eliminates remaining strep. When heart failure persists or worsens despite aggressive therapy for acute RHD, surgery to reduce valve insufficiency can be lifesaving; roughly 40% of patients with acute rheumatic fever later develop mitral stenosis as adults. Diet is unrestricted except in CHF, which calls for fluid and sodium restriction; potassium supplementation may be needed because of the mineralocorticoid effect of corticosteroids and any diuretics. Start patients on bed rest, then indoor activity before returning to school; withhold full activity until acute-phase reactants normalize. Patients with chorea may need a wheelchair and homebound instruction until the abnormal movements resolve.
Pharmacologic Management
Treatment and prevention follow American Heart Association guidelines on prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis. Antibiotics serve three roles: treat the initial GABHS pharyngitis, prevent recurrent streptococcal pharyngitis, rheumatic fever, and RHD, and provide prophylaxis against bacterial endocarditis. Anti-inflammatory agents: acute manifestations including carditis respond rapidly; aspirin in anti-inflammatory doses is the drug of choice, with prednisone added when carditis and heart failure worsen. Heart failure therapy in RHD reflects severe mitral and aortic insufficiency plus pancarditis and traditionally combines an inotrope (digitalis) with diuretics (furosemide, spironolactone) and afterload reduction (captopril).
Nursing Management
Nursing Assessment
Take a complete, current history from child and caregiver; ask about recent sore throat or upper respiratory infection, when symptoms began, the extent of illness, and any treatment. On exam, review all systems and note the child's condition, looking for major or minor manifestations: elevated temperature and pulse, erythema marginatum, subcutaneous nodules, swollen or painful joints, and signs of chorea.
Nursing Diagnoses
Based on the assessment data, the major nursing diagnoses are: acute pain related to joint pain when extremities are touched or moved; deficient diversional activity related to prescribed bed rest; activity intolerance related to carditis or arthralgia; risk for injury related to chorea; risk for noncompliance with prophylactic drug therapy related to the financial or emotional burden of lifelong therapy; and deficient caregiver knowledge related to the condition, need for long-term therapy, and risk factors.
Nursing Care Planning and Goals
The major goals are reducing pain, providing diversional activities and sensory stimulation, conserving energy, and preventing injury.
Nursing Interventions
Reduce pain by positioning the child to ease joint pain; warm baths and gentle range-of-motion exercises help, and pain-indicator scales let children express their pain level. Provide diversional activities: bed rest distresses children who do not feel very ill, so use books and quiet games that allow rest but prevent boredom, planning for the child's developmental stage. Conserve energy with rest periods between activities; if the child has chorea, tell visitors the child cannot control the movements, which upset the child as much as anyone. Prevent injury by keeping side rails up and padded, never leaving a child with chorea unattended in a wheelchair, and using all appropriate safety measures.
Evaluation
Goals are met when pain is reduced, diversional activity and sensory stimulation are provided, energy is conserved, and injury is prevented.
Documentation Guidelines
Document baseline and subsequent assessment findings including signs and symptoms; cultural or religious restrictions and personal preferences; the plan of care and persons involved; the teaching plan; responses to teaching, interventions, and actions; progress toward outcomes; and long-term needs with who is responsible for actions to be taken.
Frequently Asked Questions
What is rheumatic fever? It is a systemic, immune-mediated inflammatory disease that can follow an untreated or undertreated group A strep throat infection, affecting the heart, joints, skin, and central nervous system, most often in children aged 5 to 15.
What causes rheumatic fever? It develops only after group A beta-hemolytic streptococcal (GABHS) pharyngitis. Molecular mimicry between streptococcal proteins and human tissue triggers an autoimmune response that can damage heart valves.
How can rheumatic fever be prevented? Prompt diagnosis and a full antibiotic course for strep throat prevents most cases, and long-term antibiotic prophylaxis prevents recurrences in someone who has already had rheumatic fever (American Heart Association guidelines).
Why does rheumatic fever damage the heart? Repeated or severe inflammation scars the heart valves, leading to rheumatic heart disease (RHD). The mitral valve is affected most often (about 65 to 70 percent of patients), followed by the aortic valve.
How is rheumatic fever diagnosed? There is no single test. Clinicians apply the modified Jones criteria alongside evidence of recent strep infection (antistreptococcal antibodies), acute-phase reactants such as ESR and CRP, ECG, and echocardiography.
How serious is rheumatic heart disease worldwide? It remains a major cause of cardiovascular morbidity, with about 33.4 million prevalent cases and roughly 319,400 deaths in 2015 and a rising global burden since (Watkins et al., NEJM 2017).