A 44-year-old previously healthy woman presented to the emergency department with chest pain and shortness of breath. The patient reported a two-month history of polyarthralgias and rash. She initially noted swelling and pain of her ankles that progressed to her knees, wrists, and shoulders. The patient also reported a non-pruritic rash with erythematous borders on her extremities that would come and go (Figure 1), nodules in her skin, and intermittent fevers.
She denied any insect bites or recent travel, and had no personal or family history of autoimmune disorders. She reported a sore throat two weeks prior to onset of symptoms that was not treated.
The patient was found to be in sinus tachycardia (Figure 2), and she complained of chest pressure and difficulty breathing. She continued to have persistent tachycardia despite fluid hydration and pain control. A CT angiogram of the chest was negative for pulmonary embolism. The patient was admitted for persistent tachycardia and further workup of recent symptoms that were concerning for acute rheumatic fever.
Laboratory tests revealed a high antistreptolysin O titer. Throat culture was positive for Streptococcus pyogenes. She also had elevated ESR and CRP. An echocardiogram was done during the hospitalization, which revealed mild thickening of the mitral valve and aortic valve with mild mitral and aortic regurgitation. Echo also revealed minimal pericardial effusion.
The treating physicians felt the patient met the criteria for acute rheumatic fever (major criteria: polyarthritis, carditis/valvulitis, erythema marginatum [Figure 3], subcutaneous nodules; minor criteria: fever, arthralgia, elevated ESR and CRP, prolonged PR interval). The patient was started on penicillin 250 mg four times a day for two weeks, followed by 250 mg BID indefinitely for prophylaxis. She was also discharged on prednisone 40 mg daily and ibuprofen 800 mg TID for symptomatic relief.
The patient's arthralgias and rash improved after treatment was initiated, and she is being followed by rheumatology and cardiology as an outpatient.
Acute rheumatic fever (ARF) is a delayed sequela of group A streptococcus (GAS) pharyngeal infection. A latent period of two to three weeks precedes the first signs of ARF. ARF and subsequent rheumatic heart disease affect almost 20 million people in the developing world, and are the leading cause of cardiovascular death in patients under age 50. (Lancet Infect Dis 2005;5:685.) The mean incidence is 19 per 100,000 population. A much lower incidence of ARF is reported in the United States and developed countries, likely because of improved hygienic standard and routine antibiotic treatment of acute pharyngitis. The incidence of ARF in developed countries has been as low as two to 14 cases per 100,000, though recent local outbreaks have occurred. (Circulation 2009;119:1541; New Engl J Med 1987;316:421; Pediatr Infect Dis J 1990;9:97.)
T. Duckett Jones, MD, first described and published diagnostic criteria for acute rheumatic fever in 1944. (JAMA 1944;126:481.) The American Heart Association subsequently established guidelines for diagnosing rheumatic fever (Table 1). (JAMA 1992; 268:2069.)
A diagnosis of acute rheumatic fever requires the presence of two major manifestations or of one major and two minor manifestations with supporting evidence of a preceding group A streptococcal infection, according to the Jones criteria. Our patient had four major criteria (polyarthritis, carditis, erythema marginatum, and subcutaneous nodules) and four minor criteria (fever, arthralgia, elevated ESR and CRP, and prolonged PR interval). She also had elevated antistreptolysin O titers and a positive throat culture for group A streptococcus, both supporting a prior GAS infection.
The three major treatment goals of acute rheumatic fever are relief of symptoms, eradication of GAS, and prophylaxis against future GAS infection to prevent recurrent cardiac disease. Aspirin or other antiinflammtory agents are the mainstay of symptomatic management because of ARF, including relief of discomfort related to arthritis and fever. Oral penicillin V is the drug of choice for eradication of GAS pharyngitis. Amoxicillin and cephalexin are also acceptable treatments. Approved alternatives for patients with potential severe hypersensitivity to beta-lactam antibiotics are azithromycin, clarithromycin, and clindamycin. (Circulation 2009;119:1541.)
Prevention of recurrent GAS pharyngitis is the most effective method to limit progression of rheumatic heart disease because patients who have had rheumatic fever are at high risk for a recurrent attack. This secondary prevention requires continuous antibiotic prophylaxis, the duration of which depends on the risk of recurrence. Patients who have had rheumatic carditis are at relatively high risk for recurrence with cardiac involvement progressing with each recurrence. Prophylaxis in these patients should continue until the patient reaches age 21 or for a total of 10 years (whichever is longer). Our 44-year-old patient, who is older than the typically affected population (ages 5–15), will need prophylaxis for several years. The antibiotic of choice for prophylaxis is IM penicillin G every four weeks or PO penicillin V twice daily. (Circulation 2009;119:1541.)
Recent resurgence of rheumatic fever in the United States after years of decline suggests increases in undiagnosed and untreated cases of streptococcus pharyngitis. Rheumatic heart disease, a preventable illness, can lead to devastating complications such as cardiomyopathy, congestive heart failure, or complete heart block. It is important for physicians to remain diligent in diagnosing and treating cases of confirmed or suspected streptococcal pharyngitis.