Disease name

류마티스 열
 Rheumatic fever

Prevalence

1-9 / 100 000

Inheritance

산발성

Age of onset

소아기

ICD 10 code

I00, I01

MIM number

268240

Summary

Rheumatic fever (RF) is a multisystem inflammatory disease, which occurs as a delayed sequel to group A streptococcal pharyngitis. It may involve connective tissues of the heart, joints, skin and vessels. In developing countries, rheumatic fever is endemic and remains one the major causes of acquired cardiovascular disease. It is a major cause of mortality among subjects under 50 years of age and its annual incidence is 100-200 times greater than that observed in industrialized countries. Migratory polyarthritis with fever is frequently the initial sign. Arthritis is only present in 75% of patients. The most commonly affected joints are the knees, ankles, elbows, wrists and, far more rarely, the hips and small joints of hands and feet. The migratory character of the arthritis and the intensity of the pain are suggestive of RF, and these features are not consistently bilateral and symmetrical. Carditis occurs early (within 3 weeks of onset) and inconsistently: it is seen in 50% of cases on clinical examination and in 70% of cases by echocardiography. The carditis may appear during any bout of the disease. Endocarditis is always present and may be the most serious sequela to GABHS (Group A beta haemolytic streptococcal) infection, leading to permanent rheumatic heart disease. Treatment of RF depends on the symptomatology. Fever and joint pain/swelling are usually treated with aspirin. All patients should be treated with a ten-day course of penicillin (or alternative antibiotic in cases of allergy to penicillin). Prophylactic antibiotics to prevent further GABHS infections, which may cause a recurrence of symptoms, should be prescribed. The antibiotic treatment consists of intramuscular injection of penicillin every 3-4 weeks or bi-daily penicillin tablets. Other antibiotics are sometimes required. Antibiotics should be continued at least up to adulthood for children without carditis and for life in patients with carditis.