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Rheumatic Fever

Updated: Sep 22

MANUAL MINORS



Rheumatic fever is a multisystem inflammatory disease that occurs as a complication of a pharyngeal infection caused by Streptococcus pyogenes (group A beta-haemolytic streptococcus).


It primarily affects children and adolescents, with its most characteristic manifestation being joint inflammation (migratory arthritis), accompanied by other symptoms such as carditis, chorea (involuntary movements), erythema marginatum, and subcutaneous nodules. Rheumatic fever can lead to permanent damage to the heart valves, known as rheumatic heart disease.


Diagnosis


The diagnosis of rheumatic fever is based on the modified Jones criteria, which include major criteria (migratory arthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules) and minor criteria (fever, arthralgia, elevated inflammatory markers, and prolonged PR interval on ECG).

Additionally, evidence of a recent streptococcal infection is required, such as a positive rapid strep test, throat culture, or elevated anti-streptolysin O (ASO) antibody levels.


Differential Diagnosis

Condition

Distinctive Features

Juvenile rheumatoid arthritis

Chronic polyarthritis, no prior streptococcal infection, specific serological tests (RF, anti-CCP).

Systemic lupus erythematosus

Multisystem involvement, positive serologies (ANA, anti-DNA), malar rash.

Kawasaki disease

Prolonged fever, rash, coronary artery involvement.

Reactive arthritis

History of previous gastrointestinal or urinary infection, no cardiac involvement.

Infective endocarditis

Prolonged fever, new heart murmur, positive blood cultures, Janeway lesions, positive blood cultures.

Emergency Management


Emergency management of rheumatic fever includes administering antibiotics to eradicate the streptococcal infection (penicillin or erythromycin in case of allergy) and controlling inflammation with nonsteroidal anti-inflammatory drugs (NSAIDs) such as naproxen or ibuprofen, which help alleviate joint pain and reduce fever.


In cases of severe carditis, corticosteroids may be required. Absolute rest is recommended for patients with carditis until cardiac inflammation is controlled. Hospitalisation may be necessary in severe cases or when there is significant cardiac involvement.


Definitive Treatment


Long-term treatment includes prolonged antibiotic prophylaxis with penicillin to prevent recurrences and new streptococcal infections, which could worsen rheumatic heart disease. Patients with significant cardiac involvement may require specific treatment, including valve surgery in advanced cases. Rehabilitation and regular follow-up with cardiology are essential to manage cardiac sequelae.

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