What is the diagnosis and treatment for Acute Rheumatic Fever (ARF)?

From the Guidelines

The diagnosis of Acute Rheumatic Fever (ARF) should be made using the Revised Jones Criteria, which includes a combination of clinical features, laboratory tests, and echocardiography, as recommended by the American Heart Association in 2015 1. The diagnosis of ARF is based on the presence of two major criteria (carditis, polyarthritis, chorea, erythema marginatum, and subcutaneous nodules) or one major and two minor criteria (clinical: polyarthralgia, fever; laboratory: elevated erythrocyte sedimentation rate, elevated C-reactive protein, or prolonged PR interval), plus evidence of a preceding group A streptococcal infection. The treatment of ARF involves antibiotics to eliminate remaining streptococcal infection, typically penicillin G benzathine as a single intramuscular injection (1.2 million units for adults, 600,000 units for children under 60 pounds) or oral penicillin V (250-500 mg twice daily for 10 days) 1. Anti-inflammatory medications like aspirin (80-100 mg/kg/day divided into 4 doses for children) or corticosteroids are used to reduce inflammation and symptoms, and patients require long-term preventive antibiotic therapy, usually monthly penicillin G injections or daily oral penicillin, which may continue for 5-10 years or longer depending on heart involvement, as recommended by the American Heart Association in 2009 1. Some key points to consider in the management of ARF include:

• The duration of secondary prophylaxis depends on the number of previous attacks, the time elapsed since the last attack, the risk of exposure to GAS infections, the age of the patient, and the presence or absence of cardiac involvement 1.
• Patients with rheumatic heart disease (RHD) require lifelong prophylaxis against rheumatic fever, with the duration and type of prophylaxis depending on the severity of valve disease and exposure to group A streptococcus 1.
• Secondary prophylaxis should continue after valve surgery, and the recommended duration of prophylaxis is at least 10 years after the most recent ARF, or until 40 years of age (whichever is longer) for patients with severe RHD 1.

From the Research

Diagnosis of Acute Rheumatic Fever (ARF)

• The diagnosis of ARF is based on clinical criteria, such as the Jones criteria 2
• The Jones criteria need to be verified and revised in certain contexts, such as in the Indian context 2
• Diagnosis of group A beta-hemolytic streptococcal (GAS) pharyngitis, which can lead to ARF, is best accomplished by a throat culture 3

Treatment of Acute Rheumatic Fever (ARF)

• The treatment of ARF chiefly involves the use of antibiotics, such as penicillin, to eradicate streptococci 4, 3, 5, 2
• Anti-inflammatory drugs, such as salicylates or corticosteroids, are also used to treat the inflammatory reactions 4, 5, 2, 6
• Patients with severe carditis, congestive heart failure, and/or pericarditis are best treated with corticosteroids 4
• The treatment must be continued for 12 weeks 4, 2

Secondary Prophylaxis

• Secondary prophylaxis with antibiotics, such as penicillin, is necessary to prevent recurrences of ARF 4, 3, 5, 2
• The duration of prophylaxis depends on the number of previous attacks, the time lapsed since the last attack, the risk of exposure to streptococcal infections, the age of the patient, and the presence or absence of cardiac involvement 3
• Penicillin is the agent of choice for secondary prophylaxis, but sulfadiazine or erythromycin are acceptable alternatives in penicillin-allergic individuals 3

Management of Cardiac Complications

• The management of cardiac complications, such as valvular heart disease, congestive heart failure, and atrial fibrillation, is crucial in the treatment of ARF 4, 2
• Surgery, such as mitral valve replacement or repair, may be indicated in cases with intractable hemodynamics due to mitral regurgitation 4