Acute rheumatic fever presenting as unstable angina.

Key Words: angina, chest pain, rheumatic fever

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The most common presentation of acute rheumatic fever in adults is a febrile illness with an additive or migratory polyarthritis that is present on average in 92% of patients (range, 43-100%), or tenosynovitis usually of the wrists and ankles (67%). (1), (2) Carditis, present in 15 to 35% of patients, is usually mild and benign. (1-3) We describe a case of an adult with acute rheumatic fever who presented with chest pain mimicking unstable angina. Fever developed only later in the course of his illness.

Discussion

Rheumatic fever (RF) is an inflammatory disease of the heart, joints, central nervous system, and subcutaneous tissues that develops after a misplaying infection by group A [beta]-hemolytic streptococci. The disease was first described in the nineteenth century. There was a dramatic decline in the incidence of R in industrialized countries that is attributed to improvement in living conditions, overall socioeconomic development, and wide availability of penicillin. (4) Currently, it is a rare disease in developed countries.

The Jones criteria for guidance in the diagnosis of acute RF were first presented by T. Duchess Jones, MD, in 1944 and have been revised twice: in 1965 and in 1992. (5) If supported by evidence of a preceding group A streptococcal infection, the presence of two major manifestations or one major and two minor manifestations indicates a high probability of acute RF. Major manifestations include Carditis, ply-arthritis, chorea, erythema marginatum, and subcutaneous nodules. Minor manifestations include arthralgia, fever, elevated acute phase reactants in blood, and a prolonged PR interval on electrocardiography. Our patient satisfied one major (Carditis) and three minor (arthralgia, fever, and elevated erythrocyte sedimentation rate) Jones criteria that were supported by elevated antistreptolysin O titers.

RF is less common in adults than in children. The difference in manifestations of RF in an adult from that in a child was recognized as early as 1959. (6) In adults, there is a higher incidence of arthritis, as compared with Carditis, and an extremely low incidence of chorea, nodules, and erythema marginatum. (1), (6) Fever is present in 77% of adult patients, with ranges from 56 to 100% in different studies. (2)

Carditis in adults presents differently from children, being milder and more benign (1-3); the incidence of Carditis in adult patients with RF may vary in different parts of the world. (6) In two series, adults with rheumatic Carditis had new holosystolic murmurs and none had congestive heart failure or cardiac enlargement. (1), (2) None had pericarditis in one report, (1) and a single patient (10%) had pericarditis in the second series. (2) Only two patients with rheumatic Carditis who presented with substernal pressure-like pain suggestive of a myocardial infarction have been described in the literature. (7) Two weeks after streptococcal tonsillitis, both patients developed migratory arthralgia, fever, and pericarditic chest pain, followed by an episode of severe retrosternal pressure in each patient. The electrocardiogram during the latter episodes revealed ST elevation in the inferior leads, followed later by the appearance of pathologic Q waves in the same leads that persisted for only a few days.

Our patient presented initially without fever and with symptoms suggesting unstable angina. Fever did not develop until the third week of his illness. His electrocardiogram showed alternating right and left bundle branch blocks without PR or ST interval changes. This pattern is rare but has been described in the medical literature. (8) His atypical presentation led to a delayed diagnosis of acute RF. Clues were the patient's persistent symptoms, fever that developed on Day 18 of his illness, the development of tenosynovitis, and his job as a custodial worker in a day-care center. In the evaluation of chest pain of unclear cause, acute RF should be considered.

Key Points

* Carditis is a rare presentation of acute rheumatic fever in an adult.

* Our patient presented without fever but with chest pain as the chief complaint.

* In the evaluation of chest pain of unclear cause, acute rheumatic fever should be considered.

From the Department of Medicine, St. Vincent Catholic Medical Centers/St. Vincent's Hospital Manhattan, New York, and the Department of Medicine, New York Medical College, Valhalla, NY.

The authors have no commercial or proprietary interest in any drug, device, or equipment mentioned in this article.

Reprint requests to Larisa Litvinova, MD, Section of Infectious Disease, St. Vincent Catholic Medical Centers/St. Vincent's Hospital Manhattan, 153 W. 11th Street, Cronin 1003, New York, NY 10011. Em ail: [e-mail]

Accepted March 25, 2003.

Copyright [c] 2003 by The Southern Medical Association

0038-4348/03/9611-1154

References

1. McDanald EC, Weisman MH. Articular manifestations of rheumatic fever in adults. Ann Intern Med 1978;89:917-920.

2. Wallace MR, Garst PD, Papadimos TJ, et al. The return of acute rheumatic fever in young adults. JAMA 1989;262:2557-2561.

3. Barnert AL, Terry EE, Persellin RH. Acute rheumatic fever in adults. JAMA 1975;232:925-928.

4. Massell BF, Chute CG, Walker AM, et al. Penicillin and the marked decrease in morbidity and mortality from rheumatic fever in the United States. N Engl J Med 1988;318:280-286.

5. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. Guidelines for the diagnosis of rheumatic fever: Jones Criteria, 1992 update. JAMA 1992;268:2069-2073.

6. Mahapatra RK. The changing pattern of acute rheumatic fever. Resid Staff Physician 1982;28(Suppl):15S-21S.

7. Suliman K, Flatau E, Simon Z, et al. Rheumatic Carditis presenting as acute myocardial infarction. Isr J Med Sci 1985;21:445-450.

8. Yahalom M, Jerushalmi J, Roguin N. Adult acute rheumatic fever: A rare case presenting with left bundle branch block. Pacing Clin Electrophysiol 1990;13:123-127.

RELATED ARTICLE: Case Report

A 42-year-old black man with hypertension presented with a 2-week history of increasing substernal chest pain. The pain was sharp, intermittent, lasting 20 minutes, worsening during the prior 2 days, and associated with shortness of breath and diaphoresis. The patient had been taking acetaminophen and ibuprofen for the chest pain. He denied fever, chills, muscle weakness, and prior sore throat. The patient had a 15-pack-year smoking history and was employed as a custodial worker in a day-care center.

Initial examination revealed a slightly lethargic man with a temperature of 99.1[degrees]F, a blood pressure of 135/79 mm Hg in the right arm and 126/70 mm Hg in the left, a pulse of 85 beats/min, and an oxygen saturation of 98% while breathing ambient air. Cardiac auscultation revealed a regular rhythm with a Grade III/VI systolic murmur at the left lower sternal border. The patient was not aware of having a heart murmur in the past. Laboratory tests were significant for a hemoglobin of 11.8 g/dl, a creatinine kinase of 184 [micro]U/L (normal, 50-250 U/L), and a troponin of 5.40 ng/ml (normal, 0.00-2.00 ng/ml). His electrocardiogram revealed a left bundle branch block. An electrocardiogram obtained 2 days previously had revealed a right bundle branch block without ischemic changes. A diagnosis of unstable angina and heart block was made.

Cardiac catheterization revealed patent coronary arteries, normal left ventricular size and ejection fraction (55%), normal mitral and aortic valves, and mild anterolateral and apical hypokinesia. A temporary transvenous pacemaker was placed. The patient continued to have intermittent chest pain suggestive of acute coronary insufficiency. During the next few days, his cardiac murmur had a changing pattern, with different systolic and diastolic components at various times. He first had a temperature of 101.3[degrees]F on the fourth hospital day, which increased to 103.5[degrees]F the following morning. He complained of vague joint pains and increasing fatigue and was unable to perform simple functions such as signing his name or lifting a glass because of pain in his hands. The patient was noted to be more lethargic. He had exquisite hand tenderness bilaterally, and bilateral knee and ankle pain and tenderness. No joint swelling or rash was noted.

Bacterial blood cultures were negative for growth. Antistreptolysin O titers were elevated at 400 IU (normal, negative), erythrocyte sedimentation rate was 128 mm/h (normal, 0-15 mm/h), and C-reactive protein was 47.1 mg/dl (normal, 0-1 mg/dl). A diagnosis of acute rheumatic fever was made, and the patient was started on high-dose aspirin, 90 to 100 mg/kg/d. His fever declined within the next 48 hours with resolution of his chest pain and improvement in the fatigue and joint pains. Echo-virus (Types 4, 7, 9, 11, and 30) and coxsackievirus (Types B1-6) antibodies were negative, as were antibodies to human immunodeficiency virus.

Larisa Litvinova, MD, and Jill A. Nord, MD