Treatment
Patients with proposed ARF in acute rheumatic fever should be followed closely to ensure that the diagnosis is clear, that treatment for heart failure and other symptoms has been performed, and that measures including secondary prevention, ARF registration, and patient education have been carried out. Echocardiography should be used in all proposed cases to clarify the diagnosis and determine the severity of cardiac inflammation at baseline.
There are no treatments for ARF that can alter the likelihood or severity of future development of RHD. Treatment is symptomatic, except for heart failure treatment, which is a life-saving measure in patients with severe cardiac inflammation.
Antibiotics
All patients diagnosed with ARF need to receive adequate doses of antibiotics against group A streptococcal infections (Chapter 173). Penicillin is first and can be given orally (phenoxymethylpenicillin 500 mg, 250 mg in children less than 27 kg, Bid; or amoxicillin 50 mg/kg [maximum 1 g] daily for 10 days) or as a single injection of IM benzathine penicillin G 1.2 million units (600,000 units in children less than 27 kg).
Salicylates or NSAIDs
Once the diagnosis is established, these drugs can be used to treat arthritis, arthralgia and fever. However, they have no proven value in the treatment of cardiac inflammation or chorea. Aspirin is an optional drug at 50-60 mg/kg per day, up to a maximum of 80-100 mg/kg*d (4-8 g for adults). Monitoring for symptoms of salicylic acid toxicity, such as nausea, vomiting or tinnitus, is required when higher doses are applied. The dose should be reduced once these symptoms occur. When acute symptoms have largely resolved (most occur within 2 weeks), patients using high doses should be reduced to 50-60 mg/kg*d and continued for 2-4 weeks. Fever, joint symptoms and elevated acute chronotropic protein sometimes reappear about 3 weeks after discontinuation of the drug. This does not mean a relapse, and salicylates may be used for a short period of time. 10-20 mg/kg*d of metronidazole is an alternative to aspirin, which has the advantage of being given twice a day.
Glucocorticoids
The use of glucocorticosteroids in ARF is controversial. Two meta-analyses did not confirm that glucocorticoids were more effective than placebo or salicylic acid preparations in improving the immediate and long-term prognosis of cardiac inflammation. However, the studies included in these meta-analyses were performed 40 years ago and did not use the drugs commonly used today. Most clinicians who use glucocorticoids for the treatment of severe cardiac inflammation believe that glucocorticoids reduce acute inflammation and improve heart failure more rapidly. However, their use needs to be weighed against the potential benefits of this treatment and possible drug side effects. If applied, prednisone and prednisolone are recommended at 2-1 mg/kg*d (maximum dose 80 mg), usually for several days and up to 3 weeks.
Bed rest
The traditional recommendation of prolonged bed rest was once the cornerstone of treatment, but is no longer widely used. For patients with arthritis, arthralgia and heart failure, bed rest is used as needed. Once symptoms are well controlled, activity should be gradually resumed as tolerated by the patient.
Chorea
Medications to control abnormal movements do not alter the course or prognosis of chorea. Mild cases can usually be treated by providing a quiet environment. Severe patients require carbamazepine or sodium valproate, which are more effective than haloperidol. 1-2 weeks is often difficult to achieve and medication needs to be continued for 1-2 weeks after symptoms have resolved. Recent studies have found glucocorticoids to be effective in the treatment of chorea and to provide more rapid symptomatic relief and should be used in severe or refractory patients. Prednisone or prednisolone 0.5 mg/kg*d to start should be discontinued as soon as possible, recommended 1 week after symptom relief, with slow dose reduction or brief dose increase if symptoms worsen.
Intravenous immunoglobulin
A few studies suggest that IVIG may provide faster remission of chorea, but has not been shown to improve the short- or long-term prognosis in patients with ARF with cardiac inflammation without comorbid chorea. In the absence of better evidence, IVIG is recommended only for patients with severe chorea who have failed to respond to other treatments.
Prognosis
The average duration of disease in untreated ARF is 12 weeks. Patients are often discharged in 1-2 weeks with aggressive treatment. Inflammatory markers need to be monitored for 1-2 weeks until they are normal (mean 4-6 weeks) and UCG should be completed after 1 month to clarify any progression to cardiac inflammation. Patients with more severe cardiac inflammation require long-term close clinical and UCG monitoring.
Once the acute episode is controlled, the first priority of treatment should be to ensure long-term follow-up and compliance with secondary prevention regimens. Patients should be enrolled in the local ARF database (if available) and primary care physicians should be contacted prior to discharge to ensure follow-up plans and application of secondary prevention therapy. Patient education (including family members) should also be conducted to emphasize the importance of adherence to secondary prevention therapy.
Prevention
Primary prevention.
Ideal primary prevention must remove the major risk factors for streptococcal infection, especially overcrowded accommodation. This is often difficult to achieve in areas where ARF is common.
Therefore, the focus of primary prevention remains on primary prophylaxis (i.e., prompt and thorough treatment of sore throat due to group A streptococcal infection with antibiotics). In areas where ARF and RHD are common but group A streptococcal infections are difficult to diagnose (e.g., resource-poor countries), primary care guidelines often recommend treating all patients with sore throat with penicillin or applying a clinical management strategy to screen for people at risk for group A streptococcal pharyngitis. Although imperfect, this strategy recognizes the importance of prophylactic treatment of ARF, albeit at the cost of overtreatment of non-group A streptococcal infections for sore throat.
Secondary prevention.
The primary measure to control ARF and RHD is secondary prevention. Given that patients with ARF are more likely to develop ARF after group A streptococcal infection than the general population, they should be on long-term penicillin to prevent recurrence. The best choice for secondary prophylaxis is benzathine penicillin G (1.2 million units, 600,000 units for those ≤27 kg) injected every 4 weeks. Although more frequent dosing is rare as it can be done every 4 weeks in a population with very good compliance, it can be given every 3 weeks or even every 2 weeks in an extremely high-risk population. Oral penicillin V (250 mg) can be given twice daily as an alternative to benzathine penicillin G, but is less effective. Patients with penicillin allergy can be treated with twice daily erythromycin (250 mg).
The duration of secondary prophylaxis is influenced by a number of factors, particularly the duration of disease since the last episode (recurrence is less likely with time), age (fewer recurrences in older individuals), and the severity of RHD (if severe, even minor recurrences should be avoided because of their potentially serious consequences) (Table 381-4). Secondary prevention is best carried out as part of a collaborative RHD control program based on patient registries in each locality. Patient registries improve the ability to follow patients and identify those who are not on prophylaxis, leading to strategies to improve patient adherence.