Primary prevention
Primary prevention of rheumatic fever refers to the appropriate and timely antibiotic treatment of group A streptococcal pharyngitis and skin infections. This is recommended in populations at high risk of rheumatic fever/chronic rheumatic heart disease, and for individuals who may have personal risk factors for rheumatic fever (e.g., family history of rheumatic fever).[47] If commenced within 9 days of the onset of sore throat symptoms, intramuscular or oral administration of penicillin will usually prevent the development of acute rheumatic fever (ARF).[48][49]
Shorter courses of oral antibiotics may have comparable efficacy to standard 10-day courses of oral penicillin in treating pediatric patients with acute streptococcal pharyngitis.[50]
However, there are no data regarding the prevention of rheumatic fever with shorter courses.
A meta-analysis of studies of community-based primary prevention programs reported a reduction in risk of ARF by 59%.[51] However, only 1 of the 6 studies included in the meta-analysis was a randomized controlled trial, and this did not demonstrate a statistically significant treatment effect.[52] In New Zealand, one large-scale national rheumatic fever prevention program, centered around sore throat case finding, was implemented in 2011.[53] In one particular high-risk area, investigators observed a 58% reduction in rheumatic fever incidence among 5- to 13-year olds from 88 in 100,000 (95% CI 79 to 111) to 37 in 100,000 (95% CI 15 to 83).[54]
In low-income and middle-income countries where ARF is common, some experts assert that intensive sore throat surveillance and treatment programs cannot currently be recommended as coordinated public health programs because they have substantial cost implications.[55] However, local reports have demonstrated cost-effectiveness of primary prevention strategies, with one study also suggesting tailored approaches to reduce ARF in Africa.[56] Investigation and treatment of sore throat should continue to be promoted in settings where this strategy is feasible.
Secondary prevention
The main priority of long-term management is to ensure secondary prophylaxis is adhered to. Secondary prophylaxis is clinically effective to prevent recurrent episodes of group A streptococcal infection and is cost-effective.[122][123]
The World Health Organization (WHO) defines secondary prophylaxis for rheumatic fever as "the continuous administration of specific antibiotics to patients with a previous attack of rheumatic fever, or well-documented rheumatic heart disease (RHD). The purpose is to prevent colonization or infection of the upper respiratory tract with group A streptococci and the development of recurrent attacks of rheumatic fever."[1] Use of secondary prophylaxis in children with latent RHD (detected by echocardiography) was associated with less progression of valve lesions.[91]
The most effective antibiotic is penicillin and the most effective method of delivery of penicillin is by intramuscular injection of long-acting penicillin G benzathine every 3 to 4 weeks.[113][114]
Intramuscular penicillin G benzathine reduces streptococcal pharyngitis by 71% to 91% and reduces recurrent rheumatic fever by 87% to 96%.[114]
The internationally accepted dosage of penicillin G benzathine is the same as that for eradication of streptococci used during the acute attack.[1][124] Recommendations on the frequency of intramuscular injections and the duration of secondary prophylaxis vary between authorities. The WHO does not specify whether injections should be administered every 3 weeks or every 4 weeks. Some experts recommend injections every 3 weeks for patients at high risk (moderate to severe carditis or previous breakthrough case of acute rheumatic fever [ARF]), based on evidence that suggests that fewer recurrent episodes of ARF occur with this regimen.[125] The duration of secondary prophylaxis is determined by a number of factors, including age, time since last episode of ARF, and severity of disease. Recommended duration of prophylaxis varies across clinical guidelines.[1][63][95][117] Following growing evidence that patients with RHD who have severe valvular heart disease with or without reduced ventricular function may be dying from cardiovascular compromise following penicillin G benzathine injections, the American Heart Association (AHA) now strongly advises that patients with RHD at elevated risk receive oral antibiotic prophylaxis, preferably oral penicillin, if readily available.[115] The AHA notes that patients with elevated risk include those with severe mitral stenosis, aortic stenosis, and aortic insufficiency, those with decreased left ventricular systolic dysfunction, and those with no symptoms; for these patients, the AHA believes the risk of adverse reaction to penicillin G benzathine, specifically cardiovascular compromise, may outweigh its theoretical benefit.[115]
Patients with proven penicillin allergy should be managed with twice-daily oral erythromycin.[1] Penicillin is safe in pregnancy.[1] Intramuscular injections of penicillin G benzathine are considered sufficiently safe for anticoagulated patients.
Endocarditis prophylaxis
There is a lack of international consensus regarding the effectiveness of antibiotic prophylaxis for endocarditis. While this is not recommended in the UK, current guidelines from the American Heart Association conclude that it is reasonable to recommend antibiotic prophylaxis for individuals with certain forms of valvular heart disease (including prosthetic heart valves, annuloplasty rings) prior to dental procedures that involve manipulation of gingival tissue, manipulation of the periapical region of teeth, or perforation of the oral mucosa, for the prevention of infective endocarditis.[126][127] In Australia and New Zealand, patients with RHD have been found to have a substantially increased risk of endocarditis.[128] Australian guidelines recommend antibiotic prophylaxis if patients are having a procedure associated with a high risk of bacteremia that is associated with endocarditis and they have a cardiac condition associated with an increased risk of developing infective endocarditis and the highest risk of adverse outcomes from endocarditis.[63] New Zealand guidelines recommend antibiotic prophylaxis for those with established RHD or prosthetic valves if they are having procedures expected to produce bacteremia.[95]
Pneumococcal and influenza vaccination are recommended for patients with RHD, particularly those with severe heart involvement and heart failure.
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