Rheumatic Fever — Diagnosis and Treatment

This article is based on the guidelines provided by the AWMF, the Association of the Scientific Medical Societies in Germany.

Definition of Rheumatic Fever

Rheumatic Fever After an Infection

Rheumatic fever is an inflammatory reaction to bacterial toxins after an infection with group A streptococci.

Epidemiology of Rheumatic Fever

Distribution of Rheumatic Fever

Since in industrial countries streptococci infections of the oropharyngeal tract are usually treated with antibiotics (i.e., penicillin), this disease is very rare in these regions. In developing countries, rheumatic fever plays an important role, especially in the age of 5 to 15.

Image: “Rheumatic heart disease world map” by Lokal_Profil. License: CC BY-SA 2.5

Etiology of Rheumatic Fever

Causes of Rheumatic Fever

For example, an angina tonsillaris or pharyngitis due to streptococci A may lead to an infection-induced autoimmune reaction, which in turn can cause the rheumatic fever.

Pathophysiology of Rheumatic Fever

Rheumatic fever is a disease which is based on a cross-reaction of the produced antibodies with antigens of the sarcolemma (tropomyosin and myogen).

Clinical Presentation of Rheumatic Fever

Occurrence of Rheumatic Fever

Typically, rheumatic fever occurs two to three weeks after a declined infection. It presents as general symptoms like fever, headaches, and sweating. Also, migrating polyarthritis with swelling and reddening occurs. The heart can also be affected by rheumatic fever. Depending on which structure is affected, the disease can expand to a pancarditis. Subcutaneous rheumatic nodes can be found on the skin.

Image: “Clinical and Neuroimaging Findings of Sydenham’s Chorea.” by Ekici A, Yakut A, Yimenicioglu S, Bora Carman K, Saylısoy S. License: CC BY 2.0

A late sequela is Sydenham disease (chorea minor), which can occur months after the infection with the streptococci.

Diagnostics of Rheumatic Fever

Clinical Examination of Rheumatic Fever

The clinical examination shows a friction rub on auscultation and signs of pericardial effusion or heart insufficiency. Also, tachycardia and cardiac dysrhythmia can occur, just like audible cardiac murmurs if cardiac valves are affected.

Laboratory tests may reveal increased inflammatory parameters. Also, streptococci can be detected in the throat swab. Antibodies like antistreptolysin O or anti-desoxyribonuclease B can be determined. If the cardiac valves are affected, this can be detected in echocardiography. The ECG can demonstrate unspecific changes.

Image: “Adams-Stokes attack as the first symptom of acute rheumatic fever. Electrocardiogram showing complete A-V block with a ventricular rate of 30 bpm.” by Carano N, Bo I, Tchana B, Vecchione E, Fantoni S, Agnetti A. License: CC BY 2.0

Pathology of Rheumatic Fever

Histology of Rheumatic Fever

Image: “Incidental histological diagnosis of acute rheumatic myocarditis” by pina GS, Sampaio RO, Branco CE, Miranda GB, Rosa VE, Tarasoutchi F. License: CC BY 2.0

Histologically, so-called Aschoff bodies (image on the right) can be found, which consist of fibrinoid necroses around which rotund and giant cells have accumulated. Also, Anitschkow’s cells, which are histiocytes with owl-like nucleoli, are typical of rheumatic fever.

Differential Diagnosis of Rheumatic Fever

The rheumatoid arthritis has to be distinguished from rheumatic fever.

Therapyof Rheumatic Fever

Treatment of Rheumatic Fever

Both penicillin V and amoxicillin are suitable for treatment. A change to macrolides is possible if an allergy against penicillin is present. For treatment of the inflammation, NSAIDs are used. For prophylaxis of relapses, penicillin or a macrolide has to be administered  for at least five years.

Review Questions

Answers can be found below the references.

1. Which is usually the cause of rheumatic fever?

1. A cold.
2. A previous streptococci infection.
3. An endocarditis.
4. A myocarditis.
5. It develops idiophatically.

2. Which is most likely part of the pathological picture of rheumatic fever?

1. Osler’s nodes
2. Microabscesses with bacteria
3. Aschoff bodies
4. Serous exudate
5. Hypereosinophilia

3. Which is the first-choice treatment for rheumatic fever?

1. Penicillin
2. Macrolides
3. Lincosamides
4. Glycopeptides
5. Cephalosporins