Rheumatic fever overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Varun Kumar, M.B.B.S. [2]; Anthony Gallo, B.S. [3]

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[1]

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[2].

Diagnosis

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[3]
  • Erythema marginatum, also known as erythema annulare are pink-red rash frequently located on trunk, limbs and seldom on face[4].

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[5].

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.

Treatment

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.

References

  1. Grover A, Dhawan A, Iyengar SD, Anand IS, Wahi PL, Ganguly NK (1993). "Epidemiology of rheumatic fever and rheumatic heart disease in a rural community in northern India". Bull World Health Organ. 71 (1): 59–66. PMC 2393425. PMID 8440039.
  2. Marijon E, Ou P, Celermajer DS, Ferreira B, Mocumbi AO, Sidi D; et al. (2008). "Echocardiographic screening for rheumatic heart disease". Bull World Health Organ. 86 (2): 84. PMC 2647380. PMID 18297157.
  3. Ayoub EM (1992). "Resurgence of rheumatic fever in the United States. The changing picture of a preventable illness". Postgrad Med. 92 (3): 133–6, 139–42. PMID 1518750.
  4. BURKE JB (1955). "Erythema marginatum". Arch Dis Child. 30 (152): 359–65. PMC 2011784. PMID 13249623.
  5. Narula J, Chopra P, Talwar KK, Reddy KS, Vasan RS, Tandon R; et al. (1993). "Does endomyocardial biopsy aid in the diagnosis of active rheumatic carditis?". Circulation. 88 (5 Pt 1): 2198–205. PMID 8222115.

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