What are the guidelines for managing acute rheumatic fever in the Indian scenario?

Acute Rheumatic Fever Guidelines in the Indian Scenario

In India, secondary prophylaxis with intramuscular benzathine penicillin G 1.2 million units every 3-4 weeks is the cornerstone of acute rheumatic fever management, with the 3-week regimen showing superior outcomes in preventing recurrences and reducing progression of valvular disease. 1, 2

Diagnosis

• Diagnose acute rheumatic fever using the modified Jones criteria, which requires evidence of preceding group A streptococcal infection plus either two major criteria or one major and two minor criteria 1
• Perform throat culture or rapid antigen detection test for group A Streptococcus to confirm streptococcal pharyngitis, though testing has low sensitivity and negative results require throat culture confirmation 3
• Use echocardiography rather than auscultation alone for detecting carditis, as echocardiography is significantly more sensitive and specific for identifying valvular involvement 3

Acute Phase Treatment

Streptococcal Eradication

• Administer benzathine penicillin G 1.2 million units intramuscularly as a single dose, OR penicillin V orally, OR azithromycin for penicillin-allergic patients to eradicate group A Streptococcus 1
• Give a full therapeutic course even if throat culture is negative, as residual streptococcal organisms must be eliminated 4

Anti-inflammatory Therapy

• Use aspirin with or without corticosteroids to control the inflammatory process, continuing treatment for a total duration of 12 weeks 1
• Provide adjunctive therapy with acetaminophen or NSAIDs for moderate to severe symptoms or high fever, but avoid aspirin in children due to Reye's syndrome risk 3

Chorea Management

• Treat chorea according to severity, continuing therapy for 2-3 weeks after clinical improvement 1

Secondary Prophylaxis Regimens

First-Line Therapy

• Administer intramuscular benzathine penicillin G 1.2 million units every 3 weeks for high-risk patients (those with residual carditis or in high-prevalence areas), as this regimen demonstrates superior outcomes compared to 4-week intervals 3, 2
• The 3-week regimen reduces streptococcal infections from 12.6 to 7.5 per 100 patient-years and prophylaxis failures from 1.29 to 0.25 per 100 patient-years compared to 4-week dosing 2
• Serum penicillin levels remain adequate (≥0.02 μg/mL) in 56% of patients at 21 days versus only 33% at 28 days, explaining the superior efficacy 2

Alternative Regimens for Penicillin Allergy

• Oral penicillin V 250 mg twice daily 3, 4
• Sulfadiazine 1 g orally once daily for patients >27 kg (0.5 g for patients ≤27 kg) 3, 4
• Erythromycin orally twice daily for non-severe or immediate penicillin hypersensitivity 5
• Macrolide antibiotics (varying doses) for patients allergic to both penicillin and sulfadiazine, though avoid in patients taking cytochrome P450 3A inhibitors 3

Duration of Secondary Prophylaxis

• For rheumatic fever with carditis and residual valvular disease: Continue for 10 years after last attack OR until age 40 years, whichever is longer 3, 6, 4
• For rheumatic fever with carditis but no residual heart disease: Continue for 10 years after last attack OR until age 21 years, whichever is longer 3, 4
• For rheumatic fever without carditis: Continue for 5 years after last attack OR until age 21 years, whichever is longer 3, 4
• Consider lifelong prophylaxis for patients at high risk of group A streptococcus exposure or with severe valvular disease 3, 6
• Continue secondary prophylaxis even after valve replacement surgery, as this does not eliminate the risk of recurrent acute rheumatic fever 4

Medical Management of Cardiac Complications

Heart Failure Management

• Use guideline-directed medical therapy including diuretics, ACE inhibitors or ARBs, beta-blockers, aldosterone antagonists, and sacubitril/valsartan when left ventricular systolic dysfunction develops 3, 6
• Avoid abrupt blood pressure lowering in patients with stenotic valve lesions 3, 6
• Manage atrial fibrillation with appropriate anticoagulation for stroke prevention 3, 6

Valve Intervention Timing

• Evaluate all patients with symptomatic severe rheumatic mitral stenosis (valve area ≤1.5 cm²) for percutaneous mitral balloon commissurotomy or mitral valve surgery within 3 months of diagnosis 6
• Percutaneous mitral balloon commissurotomy is preferred for patients with favorable valve morphology (mobile, thin leaflets without significant calcium or subvalvular fusion) and <2+ mitral regurgitation without left atrial thrombus 6
• Surgical intervention is indicated when valve anatomy is unfavorable, percutaneous commissurotomy has failed, or moderate-to-severe tricuspid regurgitation requires repair 6

Infective Endocarditis Prophylaxis

• Provide antibiotic prophylaxis before dental procedures involving manipulation of gingival tissue, periapical region of teeth, or perforation of oral mucosa 6
• For patients already receiving benzathine penicillin G prophylaxis who require endocarditis prophylaxis, use amoxicillin before high-risk dental or surgical procedures 5
• If the patient has recently received penicillin or amoxicillin, or has immediate penicillin hypersensitivity, use clindamycin instead 5
• Use an agent other than penicillin for endocarditis prophylaxis in patients on penicillin prophylaxis, as oral α-hemolytic streptococci likely have developed penicillin resistance 4

Monitoring and Follow-up

• Perform echocardiographic monitoring every 3-5 years for mild disease, every 1-2 years for moderate disease, and every 6-12 months for severe disease or when the left ventricle is dilating 6
• Examine patients every 3-6 months to assess for streptococcal infections and disease progression 2
• Maintain optimal oral health as the most important component of preventing infective endocarditis 3, 4
• Administer influenza and pneumococcal vaccinations according to standard recommendations 3

Special Considerations for India

• Implement register-based comprehensive rheumatic heart disease control programs with community health worker involvement 6
• Integrate rheumatic heart disease control into primary health care systems 6
• Provide health education for patients and families about the importance of adherence to prophylaxis 6
• The 3-week benzathine penicillin regimen is particularly important in India given the high prevalence of rheumatic fever and limited healthcare access in rural areas 2

Critical Pitfalls to Avoid

• Never discontinue secondary prophylaxis prematurely, as recurrent rheumatic fever significantly worsens valvular disease 3, 6
• Do not assume that valve replacement eliminates the need for continued antibiotic prophylaxis against rheumatic fever recurrence 4
• Recognize that at least one-third of rheumatic fever cases result from asymptomatic group A streptococcal infections, so prophylaxis is essential even without symptomatic pharyngitis 7
• Avoid delaying valve intervention in symptomatic patients with severe disease, as medical therapy alone is not a substitute for definitive treatment 6
• Do not overlook pregnancy as a high-risk period requiring pre-pregnancy evaluation and potential intervention for moderate-severe rheumatic heart disease 6