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Emergency And Acute Medicine – Rheumatic Fever
Rheumatic fever is an inflammatory, autoimmune condition that follows infection with group A streptococcus (GAS), also known as Streptococcus pyogenes, most commonly after untreated streptococcal pharyngitis. It is characterized by a constellation of clinical findings defined by the Jones criteria. Although now uncommon in the United States, it remains a major cause of cardiac morbidity and mortality worldwide, accounting for over 230,000 deaths annually. It most commonly affects children between 5 and 15 years of age and is more prevalent in developing nations and in populations living in crowded or resource-limited conditions.
The disease results from an abnormal immune response to GAS infection. Molecular mimicry between streptococcal antigens and human tissues—particularly cardiac, synovial, skin, and neural tissue—leads to cross-reactive antibodies and inflammatory injury. Symptoms typically develop 2 to 4 weeks after the initial streptococcal infection.
Diagnosis requires either two major Jones criteria or one major and two minor criteria, along with evidence of a recent GAS infection. Major manifestations include migratory polyarthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules. Migratory polyarthritis occurs in 60–75% of initial attacks and typically involves large joints such as the knees, ankles, hips, elbows, and wrists, with lower extremities more commonly affected. The arthritis responds well to salicylates. Carditis occurs in one-third to one-half of new cases and may involve the pericardium, myocardium, and endocardium (pancarditis). Clinical signs include a new murmur, tachycardia, gallop rhythm, pericardial friction rub, or congestive heart failure. Valvular involvement—particularly of the mitral and aortic valves—is the most serious long-term complication. Chorea (Sydenham chorea) occurs in about 10% of cases and predominantly affects teenage girls. It is characterized by purposeless, uncoordinated movements that worsen with stress and disappear during sleep. Chorea may be the sole manifestation of the disease. Erythema marginatum is a nonpruritic rash with central clearing and irregular borders, typically seen on the trunk and extremities. Subcutaneous nodules are painless and found over extensor surfaces.
Minor manifestations include fever above 38°C, arthralgia, elevated acute phase reactants (such as ESR or C-reactive protein), and prolonged PR interval on ECG. Evidence of recent GAS infection may include a positive throat culture or rapid antigen test, or elevated or rising antistreptolysin O (ASO) titers.
Patients often report a recent sore throat, fever, joint pain, rash, unusual movements, dyspnea, or edema. Physical examination may reveal pharyngeal erythema, rash consistent with erythema marginatum, subcutaneous nodules, new heart murmurs (commonly mitral regurgitation), or signs of fluid overload and heart failure.
The essential workup includes careful cardiac and joint examination, throat swab for rapid strep testing or culture, ECG, chest radiograph, and echocardiogram. Laboratory studies include CBC, ESR or C-reactive protein, ASO titer, and other serologic tests to exclude alternative rheumatologic conditions. Diagnosis remains clinical, based on meeting the Jones criteria.
The differential diagnosis includes juvenile idiopathic arthritis, infective endocarditis, systemic lupus erythematosus, postgonococcal arthritis, Reiter syndrome, and other infectious causes of arthritis and carditis such as Coxsackie B virus or parvovirus. Rheumatic fever is primarily a pediatric disease, though it can occur in young adults. Testing for streptococcal pharyngitis is generally not recommended in children under 3 years of age in the United States because both strep throat and rheumatic fever are rare in this age group. Women with a history of rheumatic fever should receive prenatal counseling due to increased cardiac risks during pregnancy.
Management focuses on eradication of streptococcal infection, control of inflammation, and treatment of complications. Penicillin is the treatment of choice, administered intramuscularly, intravenously, or orally. Patients allergic to penicillin may receive alternatives such as azithromycin or erythromycin. Aspirin is used for arthritis and carditis, while corticosteroids such as prednisone may be required in severe carditis. Haloperidol may be used for severe chorea. Patients with heart failure may require diuretics such as furosemide and, in some cases, digoxin. Pericardial effusions may require drainage.
Most patients with a new diagnosis should be admitted for evaluation and stabilization, especially if they have congestive heart failure, uncontrolled chorea, pericardial effusion, severe pain, or significant carditis. Discharge is appropriate once pain is controlled, cardiovascular status is stable, and reliable follow-up is ensured. All patients require long-term secondary prophylaxis with monthly intramuscular benzathine penicillin to prevent recurrence, often for years depending on cardiac involvement.
Close follow-up with a cardiologist is essential for echocardiographic monitoring and guidance regarding endocarditis prophylaxis. Infectious disease consultation may be helpful for management of prolonged antibiotic prophylaxis, and rheumatology referral may be considered for persistent joint symptoms.
Although uncommon in the United States, rheumatic fever remains an important preventable cause of valvular heart disease worldwide. Prompt recognition and treatment of streptococcal pharyngitis remain critical to preventing recurrence and long-term cardiac complications.
Rheumatic fever is an inflammatory, autoimmune condition that follows infection with group A streptococcus (GAS), also known as Streptococcus pyogenes, most commonly after untreated streptococcal pharyngitis. It is characterized by a constellation of clinical findings defined by the Jones criteria. Although now uncommon in the United States, it remains a major cause of cardiac morbidity and mortality worldwide, accounting for over 230,000 deaths annually. It most commonly affects children between 5 and 15 years of age and is more prevalent in developing nations and in populations living in crowded or resource-limited conditions.
The disease results from an abnormal immune response to GAS infection. Molecular mimicry between streptococcal antigens and human tissues—particularly cardiac, synovial, skin, and neural tissue—leads to cross-reactive antibodies and inflammatory injury. Symptoms typically develop 2 to 4 weeks after the initial streptococcal infection.
Diagnosis requires either two major Jones criteria or one major and two minor criteria, along with evidence of a recent GAS infection. Major manifestations include migratory polyarthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules. Migratory polyarthritis occurs in 60–75% of initial attacks and typically involves large joints such as the knees, ankles, hips, elbows, and wrists, with lower extremities more commonly affected. The arthritis responds well to salicylates. Carditis occurs in one-third to one-half of new cases and may involve the pericardium, myocardium, and endocardium (pancarditis). Clinical signs include a new murmur, tachycardia, gallop rhythm, pericardial friction rub, or congestive heart failure. Valvular involvement—particularly of the mitral and aortic valves—is the most serious long-term complication. Chorea (Sydenham chorea) occurs in about 10% of cases and predominantly affects teenage girls. It is characterized by purposeless, uncoordinated movements that worsen with stress and disappear during sleep. Chorea may be the sole manifestation of the disease. Erythema marginatum is a nonpruritic rash with central clearing and irregular borders, typically seen on the trunk and extremities. Subcutaneous nodules are painless and found over extensor surfaces.
Minor manifestations include fever above 38°C, arthralgia, elevated acute phase reactants (such as ESR or C-reactive protein), and prolonged PR interval on ECG. Evidence of recent GAS infection may include a positive throat culture or rapid antigen test, or elevated or rising antistreptolysin O (ASO) titers.
Patients often report a recent sore throat, fever, joint pain, rash, unusual movements, dyspnea, or edema. Physical examination may reveal pharyngeal erythema, rash consistent with erythema marginatum, subcutaneous nodules, new heart murmurs (commonly mitral regurgitation), or signs of fluid overload and heart failure.
The essential workup includes careful cardiac and joint examination, throat swab for rapid strep testing or culture, ECG, chest radiograph, and echocardiogram. Laboratory studies include CBC, ESR or C-reactive protein, ASO titer, and other serologic tests to exclude alternative rheumatologic conditions. Diagnosis remains clinical, based on meeting the Jones criteria.
The differential diagnosis includes juvenile idiopathic arthritis, infective endocarditis, systemic lupus erythematosus, postgonococcal arthritis, Reiter syndrome, and other infectious causes of arthritis and carditis such as Coxsackie B virus or parvovirus. Rheumatic fever is primarily a pediatric disease, though it can occur in young adults. Testing for streptococcal pharyngitis is generally not recommended in children under 3 years of age in the United States because both strep throat and rheumatic fever are rare in this age group. Women with a history of rheumatic fever should receive prenatal counseling due to increased cardiac risks during pregnancy.
Management focuses on eradication of streptococcal infection, control of inflammation, and treatment of complications. Penicillin is the treatment of choice, administered intramuscularly, intravenously, or orally. Patients allergic to penicillin may receive alternatives such as azithromycin or erythromycin. Aspirin is used for arthritis and carditis, while corticosteroids such as prednisone may be required in severe carditis. Haloperidol may be used for severe chorea. Patients with heart failure may require diuretics such as furosemide and, in some cases, digoxin. Pericardial effusions may require drainage.
Most patients with a new diagnosis should be admitted for evaluation and stabilization, especially if they have congestive heart failure, uncontrolled chorea, pericardial effusion, severe pain, or significant carditis. Discharge is appropriate once pain is controlled, cardiovascular status is stable, and reliable follow-up is ensured. All patients require long-term secondary prophylaxis with monthly intramuscular benzathine penicillin to prevent recurrence, often for years depending on cardiac involvement.
Close follow-up with a cardiologist is essential for echocardiographic monitoring and guidance regarding endocarditis prophylaxis. Infectious disease consultation may be helpful for management of prolonged antibiotic prophylaxis, and rheumatology referral may be considered for persistent joint symptoms.
Although uncommon in the United States, rheumatic fever remains an important preventable cause of valvular heart disease worldwide. Prompt recognition and treatment of streptococcal pharyngitis remain critical to preventing recurrence and long-term cardiac complications.
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