Rheumatic fever overview


 * Associate Editor(s)-in-Chief:

Overview
Rheumatic fever is a systemic immune disease that may develop after an infection with streptococcus bacteria, such as strep throat and scarlet fever. The disease can affect the heart, joints, blood vessels, and brain. Usual symptoms include fever, joint apin, joint swelling, skin nodules, skin rash, epistaxis, even cardiac problems such as shortness of breath, chest pain, and emotion changes. Medical history and physical examination are very important for diagnosis. Antistreptolysin O (ASO) titer can assist in making a diagnosis of rheumatic fever. Treatments include antibiotics to control streptococcus infection and medications such as aspirin and corticosteroids to decrease inflammatory. A long-lasting injection of penicillin is important and effective to prevent further complications and recurrence.

Epidemiology and Demographics
Rheumatic fever, and therefore Streptococus pyogenes infections, are endemic in many developing countries. In countries affected by the industrial revolution, domestic living conditons became less crowded, due to the development of larger homes and families had fewer children. In addition, living conditions became, generally, more hygienic. The introduction of antibiotics, first sulfonamide in the early 1930's and then penicillin in the 1940's, further caused Streptococcus pyogenes infections to become less common and less severe in economically developed countries although they never disappeared. Rheumatic fever is usually seen among children belonging to age group of 5-15 years

Screening
Screening of rheumatic fever and carditis is important as many cases of RHD are subclinical. Echocardiography in inhabitants of high risk regions is recommended. If any abnormality is detected on echocardiography, further cardiac evaluation is done followed by prophylactic treatment.

Physical Examination

 * Fever
 * Cardiac murmurs may be noted on cardiac auscultation if heart valves are involved. Regurgitant murmurs are common.
 * Sydenham's chorea
 * Migratory polyarthritis may be noted in 70-75% of patients. Often large joints of lower limbs (knee and ankle joints) and upper limbs (elbow and wrist joints) are involved progressing below-upwards.
 * Subcutaneous nodules over extensor surface of limbs, bony prominences such as elbows, knees, ankles and knuckles
 * Erythema marginatum, also known as erythema annulare are pink-red rash frequently located on trunk, limbs and seldom on face.

Lab Tests
Patients with rheumatic fever often have elevated inflammatory markers such as ESR and C-reactive protein which help in monitoring the course of the disease. Presence of streptococcal infection can be established by obtaining throat culture or rapid streptococcal antigen test. Elevated or rising antistreptolysin O antibody titer is often noted. Endomyocardial biopsy demonstrate the presence of Aschoff bodies. However, biopsy is not recommended routinely.

Electrocardiogram
Some of the electrocardiographic changes that may be noted in rheumatic heart disease include PR prolongation, conduction abnormalities, arryhthmias or P mitrale depending on the structures involved and the extent of cardiac damage.

Chest X Ray
Cardiomegaly or pulmonary edema secondary to heart failure may be noted on chest x-ray among patients with rheumatic heart disease.

Echocardiography
Echocardiography may be helpful in establishing carditis and in monitoring the progress of valve defect.

Primary Prevention
Treatment of streptococcal pharyngitis with appropriate antibiotics (penicillin or cephalosporin) most often prevents development of rheumatic fever.

Secondary Prevention
In order to prevent recurrent development of rheumatic fever, an antibiotic prophylaxis should be initiated immediately after the antibiotic course in treatment of rheumatic fever. Duration of prophylactic treatment varies with degree of cardiac damage secondary to rheumatic fever.