Rheumatic fever is rare in the industrialized countries of the world, and is more common in Asia, Africa and South America. It usually affects children between the ages of 5 and 15 years, or young adults.
The disease is more common among socially and economically disadvantaged populations. It is important to detect and treat rheumatic fever early and adequately because it can lead to heart disease in childhood and adolescence and is the most common cause of it.
Rheumatic fever is caused by an abnormal reaction by the person to infection of tonsils and throat by the bacteria group A streptococci. The body produces antibodies to the bacterial antigens, which leads to inflammation of heart, joints and skin.
Symptoms and Signs of Rheumatic Fever
Usually 2 to 3 weeks after astreptococcal throat infection or sore throat, the patient will have fever, lethargy, anorexia and joint pains. Almost 2/3rds of the patients will have arthritis (inflammation of the joints). Other features are skin rashes, carditis (inflammation of the heart) and neurological changes.
Carditis may present as breathlessness (indicating heart failure or pericardial effusion), chest pain or palpitations. Other features include tachycardia (high pulse rate), cardiac enlargement, and heart murmurs (abnormal sounds made by the heart, which the doctor listens for with a stethoscope). Changes can also occur on the ECG such as ST and T wave changes.
In one-third of the cases, neurological changes occur (Sydenham’s chorea or St Vitus dance) characterized by emotional lability, purposeless involuntary movements of the hands, feet or face. Speech may be halting and explosive. In a few months spontaneous recovery can occur, but in one-quarter of patients with Sydenham’s chorea heart valves get affected.
Arthritis occurs early, involving usually the large joints (knees, ankles, elbows and wrists). The joints are involved in succession (migratory polyarthritis), and are usually red, swollen, painful (arthralgia) and tender for between a day and a week. Aspirin provides relief.
Skin rashes (erythema marginatum) occur in less than 10% of the patients, on the trunk and limbs, sparing the face. Nodules below the skin (subcutaneous nodules) occur in a few patients and are 0.5 to 2 cm in size, firm and painless. They usually occur after 2 to 3 weeks after other signs and symptoms appear.
Diagnosis of Rheumatic Fever
The diagnosis is usually made clinically on the basis of symptoms and signs, with supporting evidence from lab tests. Because the symptoms and signs vary from patient to patient, Jones proposed certain criteria in 1944, to standardize the diagnosis of rheumatic fever, known as Jones Criteria.
They are divided into major and minor criteria, with either 2 major criteria, or 1 major and 2 minor criteria, plus evidence of a recent streptococcal infection required for diagnosis. The major criteria are carditis, migratory polyarthritis, Sydenham’s chorea, subcutaneous nodules and erythema marginatum. The minor criteria are fever, arthralgia, and 2 laboratory criteria.
Treatment is with penicillin, aspirin and corticosteroids. Long-term prophylaxis with penicillin is needed to prevent future attacks.
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