Rheumatic Fever

Rheumatic fever does not present with specific signs and symptoms. There is sudden onset of fever, joint pain, malaise and loss of appetite. In some cases, epistaxis and abdominal pain may also be present.

The diagnosis is made with the help of Duckett Jones criteria [5]. The presence of two major criteria; or one major and two minor criteria is diagnostic.

The major criteria include the following:

• Carditis: Carditis is suggested by the presence of a number of signs that include sinus tachycardia, murmurs of mitral or aortic regurgitation, Carey-Coomb’s murmur, pericardial friction or rub, congestive heart failure, cardiomegaly and ectrocardiographic abnormalities.
• Polyarthritis: Migratory polyarthritis is present in up to 75% of the cases and mainly involves large joints such as knees, ankles, elbows and wrists.
• Syndenham’s chorea: Syndenham’s chorea develops in less than 10% of the patients suffering from rheumatic fever. However, when it is present, it is the most diagnostic feature of rheumatic fever.
• Erythema marginatum: Pink rashes with slightly raised edges may develop on the trunk or limbs.
• Subcutaneous nodules: Small, hard, painless nodules may form under the skin.

The minor criteria include the following:

• Fever
• Arthralgia
• Previous history of rheumatic fever
• Raised Erythrocyte Sedimentation Rate (ESR) or C-reactive protein (CRP)
• Leukocytosis
• Prolonged PR interval on electrocardiogram

The diagnosis of rheumatic fever is usually clinical. The following investigations may be helpful.

• Throat swab culture: This is done to look for group A streptococci.
• Anti-streptolysin O titer (ASOT): This indicates a recent streptococcal infection and is raised in up to 80% of the cases [6] [7].
• Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP): These indicate active inflammation and are raised in the acute phase of the disease.
• Blood tests: Leukocytosis is seen with a total of around 12000 to 15000 white cells per cubic millimeter. Mild to moderate normocytic, normochromic anemia may be present.
• Chest X-ray: Chest X-ray will either be normal or will show signs of cardiomegaly, pulmonary edema and/or increased pulmonary vascularity.
• Electrocardiography (ECG): There may be prolonged PR interval, heart block or features of pericarditis and myocarditis.
• Echocardiography: Mitral regurgitation, myocardial dysfunction and pericardial effusion may be seen.

The treatment of rheumatic fever has the following components [8] [9].

Bed rest: The patient should be advised complete bed rest until the temperature, resting pulse, erythrocyte sedimentation rate (ESR) and electrocardiogram (ECG) return to normal.

Aspirin: Aspirin and other salicylates are very effective in reducing the fever and relieving joint pain and swelling in the patients suffering from rheumatic fever. Aspirin therapy is continued for a period of 2 weeks. If the polyarthritis is controlled by then, the dosage is tapered for an additional 6 weeks. This is necessary because sudden discontinuation of aspirin therapy can cause recurrence of symptoms.

Corticosteroids: If the response to aspirin therapy is inadequate or if there is severe arthritis or carditis, a short course of steroids is given for 2 weeks after which the dosage is tapered over a period of 3 weeks.

During the initial phase, symptoms may last for several months in children and several weeks in adults. The mortality rate in this phase is 1 to 2%.

Valve disease occurs in up to two thirds of the patients by 10 years; however, not all the patients will develop symptoms or cardiomegaly.

Poor prognosis is implied if the patient develops persistent rheumatic carditis associated with cardiomegaly, heart failure or pericarditis.

Rheumatic fever usually follows a pharyngeal infection caused by beta-hemolytic streptococcal species after a latent period of approximately 3 weeks. It results from the cross-reaction of the body’s immune response to the streptococcal antigen with its own tissues - principally those of the heart [1] [2].

Rheumatic fever is much more prevalent in the developing countries where the incidence is as high as 1 case per 1000 population. In the developed countries, factors such as improved hygiene and living conditions, decreased crowding, the use of antibiotics and proper treatment have greatly reduced the incidence of rheumatic fever [3] [4].

Rheumatic fever commonly occurs in children aged 5 to 15 years - the peak age being 8 years. It is rare before the age of 4 and occasional cases are seen after the age of 30.

The acute phase of rheumatic fever is characterized by exudative and proliferative inflammatory reactions involving the heart, joints, brain, skin and subcutaneous tissues.

Rheumatic carditis principally involves the mitral (75-80%) and aortic (30%) valves. The valve cusps become thickened by edema and by infiltration of capillaries. Later on, a row of vegetations forms along the lines of closure of the valve leaflets. Inflammation of the valves leads to mitral and aortic regurgitation. In addition to valvulitis, there may be myocarditis and pericarditis.

Primary prevention: The development of rheumatic fever can be stopped if there is prompt recognition and proper treatment for group A streptococcal pharyngitis and tonsillitis. Intramuscular benzathine penicillin is the antimicrobial agent of choice. In the patients allergic to penicillin, erythromycin or azithromycin may be used [10].

Secondary prevention: The recurrence of rheumatic fever can be prevented by continuous antimicrobial prophylaxis. An injection of benzathine penicillin should be given every 4 weeks. Salphasalazine or erythromycin are given in the patients who can not tolerate penicillin [11].

Rheumatic fever is an acute inflammatory disease that follows infection with group A beta-hemolytic streptococci. It is characterized by inflammatory lesions of connective tissue, mainly the heart, blood vessels and joints.

The patients may develop carditis, polyarthritis, Syndenham’s chorea, erythema marginatum and subcutaneous nodules. In addition, there may be fever, abdominal pain and other non-specific features.

Rheumatic fever occurs as a delayed sequela of throat infection with certain species of bacteria. It occurs much more commonly in children as compared to adults. The disease affects many parts of the body, in particular the heart and the joints. If the throat infection with the causal bacteria is detected and treated early, rheumatic fever can be prevented. The treatment of rheumatic fever is mostly symptomatic. Antibiotics are used to prevent the disease from occurring after it has been successfully controlled.

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