Rheumatic Diseases and Recurrent Infections
Yes, acute rheumatic fever (ARF) is a rheumatic disease that characteristically presents with recurrent episodes following repeated Group A streptococcal infections, and patients with a history of ARF are at particularly high risk for recurrent attacks if reinfected. 1
Understanding the Recurrence Pattern
Acute rheumatic fever is fundamentally linked to recurrent Group A streptococcal infections, making it unique among rheumatic diseases in its infectious trigger and recurrence pattern. The disease occurs as an autoimmune response to pharyngitis caused by Group A Streptococcus, and patients who have had one episode face substantially elevated risk for subsequent episodes with each new streptococcal infection. 2
Key Clinical Features of Recurrence:
Patients with prior ARF or rheumatic heart disease (RHD) are at high risk for recurrent attacks when reinfected with Group A streptococci, though each recurrence may not fulfill complete Jones criteria. 1
At least one-third of rheumatic fever cases result from asymptomatic Group A streptococcal infections, making prevention particularly challenging since patients may develop recurrences without obvious pharyngitis symptoms. 3, 4
Each recurrence carries risk of progressive cardiac damage, with patients who have had rheumatic carditis being especially prone to increasingly severe cardiac involvement with subsequent attacks. 1
Diagnostic Approach for Recurrences
When evaluating suspected ARF recurrence in a patient with known history:
With documented Group A streptococcal infection and reliable past history of ARF/RHD: 2 major manifestations, OR 1 major plus 2 minor manifestations, OR 3 minor manifestations may be sufficient for presumptive diagnosis. 1
When only minor manifestations are present: Exclude other more likely causes before diagnosing ARF recurrence. 1
In patients with recurrent joint symptoms who are adherent to prophylaxis but lack serological evidence of streptococcal infection and lack echocardiographic valvulitis: The symptoms are likely not ARF-related, and discontinuation of prophylaxis may be appropriate. 1
Critical Management: Secondary Prophylaxis
The cornerstone of preventing recurrent ARF is continuous antimicrobial prophylaxis, not episodic treatment of acute infections. 1, 4
Preferred Prophylaxis Regimen:
Intramuscular benzathine penicillin G: 1.2 million units every 4 weeks is the recommended regimen for most circumstances. 1, 3
Every 3-week administration is justified in high-incidence populations or patients with recurrences despite adherence to the 4-week regimen. 1, 3
Alternative Regimens:
Oral penicillin V: 250 mg twice daily for those unable to receive intramuscular injections. 1
Sulfadiazine: 1 g orally once daily as an alternative. 1
Macrolide or azalide antibiotics for patients allergic to both penicillin and sulfadiazine (avoid with cytochrome P450 3A inhibitors). 1
Duration of Prophylaxis
The duration depends on cardiac involvement and must be individualized based on specific risk factors:
Rheumatic carditis with residual heart disease: 10 years after last episode OR until age 40 (whichever is longer), potentially lifelong for high-risk patients. 1, 3
Rheumatic carditis without residual heart disease: 10 years OR until age 21 (whichever is longer). 1, 3
Rheumatic fever without carditis: 5 years OR until age 21 (whichever is longer). 1
High-Risk Factors Warranting Extended Prophylaxis:
- Children and adolescents 1
- Parents of young children 1
- Teachers and healthcare personnel in contact with children 1
- Military recruits 1
- Those in crowded living situations (college dormitories) 1
- Economically disadvantaged populations 1
Critical Pitfalls to Avoid
Prophylaxis must continue even after valve surgery, including prosthetic valve replacement. 1 This is a commonly missed detail that can lead to devastating recurrences.
Even when streptococcal pharyngitis is treated optimally, rheumatic fever can still occur in susceptible individuals, emphasizing that treatment of acute infections alone is insufficient—continuous prophylaxis is essential. 3, 4
Infection does not have to be symptomatic to trigger recurrence, so patients cannot rely on recognizing and treating symptomatic pharyngitis as their prevention strategy. 1
Other Infectious Mimics of Rheumatic Disease
While ARF is the primary rheumatic disease with recurrent infections as a core feature, be aware that numerous infections can mimic rheumatic diseases including:
- Viral infections (Parvovirus B19, Hepatitis B/C, Chikungunya, HIV) 5, 6
- Mycobacterial infections (Poncet's disease, tubercular arthritis) 6
- Lyme disease and brucellosis 6
These infectious mimics are critical to exclude before initiating immunosuppressive therapy for presumed primary rheumatic disease, as immunosuppression can aggravate underlying infections. 6