Approach
No treatments affect the outcome of acute rheumatic fever (ARF). While treatment can shorten the acute inflammation, all of the various manifestations will resolve spontaneously, except for carditis. The degree of valvular regurgitation usually improves but may occasionally progress in the months following a diagnosis of rheumatic fever. No treatment used in the acute phase is effective at modifying the acute course of carditis, and good adherence with secondary prophylaxis is the only treatment known to ameliorate the long-term progression to rheumatic heart disease (RHD); the major role of secondary prevention after ARF is prevention of rheumatic fever recurrences and cumulative subsequent repeat valve damage.[96]
Aims of acute management
The aims of management are to:
Confirm the diagnosis of ARF
Provide symptomatic treatment and shorten the acute inflammatory phase, particularly polyarthritis, which can be very painful
Provide education for the patient and the patient's family
Begin secondary prophylaxis and emphasize its importance
Offer dental treatment and education regarding prevention of infective endocarditis
Ensure follow-up.
All patients with suspected ARF should be admitted to the hospital so diagnosis can be confirmed, and clinical features and severity of the attack can be assessed. Some patients with a confirmed diagnosis and mild illness may be managed as outpatients after an initial period of stabilization.
Unconfirmed diagnosis, presenting with monoarthritis
If ARF is suspected, admission to the hospital for observation and further investigation is indicated. Nonsteroidal anti-inflammatory drugs (NSAIDs) and salicylates such as aspirin should be withheld pending confirmation of the diagnosis, and simple analgesics, such as acetaminophen, are recommended in the interim. NSAIDs and aspirin are withheld to facilitate diagnosis; they reduce arthritic pain but do not affect the long-term outcome of the disease. For example, if there is doubt about the diagnosis in a patient with monoarthritis, the appearance of migratory polyarthritis will confirm rheumatic fever. A preferable strategy in patients with suspected rheumatic fever is bed rest and regular acetaminophen while performing investigations to confirm the diagnosis as quickly as possible. Once the diagnosis is confirmed, start the patient on regular NSAIDs.
Opioid analgesics are generally not recommended, especially in children. They should be avoided wherever possible. If they are necessary, they should be used with extreme caution. Codeine is contraindicated in children younger than 12 years of age, and it is not recommended in adolescents 12 to 18 years of age who are obese or have conditions such as obstructive sleep apnea or severe lung disease as it may increase the risk of breathing problems.[97] It is generally recommended only for the treatment of acute moderate pain, which cannot be successfully managed with other analgesics, in children 12 years of age and older. It should be used at the lowest effective dose for the shortest period and treatment limited to 3 days.[98][99]
Confirmed ARF
The outcome of rheumatic valvular lesions has not been shown to be affected by the administration of penicillin during an episode of ARF.[100] However, penicillin is recommended to ensure eradication of streptococci in the throat whether there has been a positive throat culture or not. A single injection of penicillin G benzathine is the recommended primary option. Oral penicillin is an option if intramuscular penicillin is refused; however, adherence needs to be closely monitored. Intravenous penicillin is not necessary. In general, penicillin G benzathine is recommended as this also serves the purpose of acting as the first dose of secondary prophylaxis. Oral erythromycin is an option for patients allergic to penicillin and adherence should be closely monitored. As penicillin is the first-line choice for secondary prophylaxis, it is recommended that a patient with reported penicillin allergy be carefully evaluated. Referral to an allergist may be necessary and desensitization may be appropriate in some circumstances, under careful medical supervision. Education is a second key component of the acute management of all patients with rheumatic fever because of the necessity of ongoing long-term preventive measures.
Particular management approaches are needed for the three main manifestations of ARF.
Arthritis
Carditis
Chorea
With arthritis
Historically, the first-line treatment for rheumatic arthritis has been salicylate therapy (aspirin).[57][101][102][103] Increasingly, clinicians are recommending NSAID treatment. Naproxen has been used successfully, and some experts recommend naproxen as first-line treatment because of its twice-daily dosing, superior side-effect profile, and reduced risk of Reye syndrome.[104][105]
Ibuprofen has also been successfully used in children, although there are no specific data regarding its effectiveness in children with rheumatic fever.[63]
If the patient has monoarthritis and is suspected to have ARF, but does not meet the criteria for diagnosis, the patient should withhold from salicylate therapy or NSAID treatment so that the appearance of migratory polyarthritis (a major manifestation) is not masked. Acetaminophen can be given in the interim.
Aspirin and NSAIDs usually have a dramatic effect on the arthritis and fever of rheumatic fever, and typically this improves within 2 to 3 days after the initiation of regular treatment. Persisting rheumatic arthritis can occasionally occur, usually this is in children with ongoing significant systemic inflammation.
With carditis
Most patients with mild or moderate carditis without cardiac failure are asymptomatic and do not require specific cardiac medications.
A subset of patients with carditis who develop cardiac failure do require treatment:
Bed rest with ambulation as tolerated
Medical management of heart failure; first-line therapy consists of diuretics and angiotensin-converting enzyme (ACE) inhibitors.
Despite the absence of high-quality evidence to support the use of glucocorticoid therapy for patients with severe carditis and heart failure, many clinicians treating rheumatic fever think that the use of glucocorticoids can speed recovery. Two meta-analyses have failed to show any benefit of glucocorticoids over placebo, although contributing studies were old and generally of low quality.[96][106]
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There is no evidence that salicylates or intravenous immunoglobulin (IVIG) improve the outcome from carditis in rheumatic fever and their use is not recommended.[107] Rarely, a patient with atrial fibrillation may require anti-arrhythmic treatment with amiodarone or digoxin.
Carditis can progress over weeks to months, and serial echocardiography is recommended for individuals with severe carditis, changing murmurs, and/or persistent inflammation.
For patients with severe carditis requiring surgery, surgery is deferred wherever possible, until systemic inflammation has settled. In rare circumstances, urgent and life-saving surgery may be necessary for complications such as acute valve leaflet rupture or chordae tendineae rupture.
Wherever feasible, cardiac valve repair is the preferred surgical procedure for established RHD, as it spares the patient the considerable additional risks associated with lifelong anticoagulation. Repair may not be possible in some situations where valvular tissues are extremely friable or severely chronically damaged.
With chorea
Most patients with chorea do not require treatment, as chorea is benign and self-limiting. Most symptoms resolve within weeks and almost all within 6 months. Reassurance and a quiet and calm environment often suffice.
Treatment is reserved for severe chorea that puts the person at risk of injury or is extremely disabling or distressing. Valproic acid or carbamazepine may be used. Valproic acid may be more effective than carbamazepine, but carbamazepine is preferred as first-line treatment because of the potential for liver toxicity with valproic acid.[63][108] Valproic acid and its derivatives may cause major congenital malformations, including neurodevelopmental disorders and neural tube defects, after in utero exposure. These agents must not be used in female patients of childbearing potential unless other options are unsuitable, there is a pregnancy prevention program in place, and certain conditions are met. Precautionary measures may also be required in male patients owing to a potential risk that use in the 3 months leading up to conception may increase the likelihood of neurodevelopmental disorders in their children. Regulations and precautionary measures for female and male patients may vary between countries and you should consult your local guidance for more information.
Treatment may ameliorate symptoms of chorea completely, or simply reduce them. Usually it takes 1-2 weeks for medication to have an effect, and it is recommended that treatment continue for 2-4 weeks after chorea has subsided. Haloperidol and combinations of agents should be avoided.
A number of small studies have suggested that IVIG treatment can allow a more rapid recovery from rheumatic chorea.[109][110] However, until further evidence is available, IVIG should not be considered as standard treatment and should be reserved for consideration in patients with severe chorea that is refractory to other treatments.
Secondary prophylaxis
The role of secondary prophylaxis is to prevent further group A streptococcal infections and recurrent episodes of overt or silent rheumatic fever leading to worsening of heart valve damage. Although some mild cases of carditis do heal completely, the progression to severe RHD is poorly understood. Secondary prophylaxis may reduce the clinical severity and thus mortality of RHD and lead to regression of RHD if patients are adherent over a decade.[111][112] The most effective antibiotic is penicillin and the most effective method of delivery is intramuscular injection of long-acting penicillin G benzathine every 3-4 weeks.[113][114] Patients with proven penicillin allergy can be managed with oral erythromycin.[1]
In patients with possible rheumatic fever (i.e., patients, generally in high-incidence settings, in whom the clinician is highly suspicious of the diagnosis of ARF but who do not quite meet the Jones criteria, perhaps because full testing facilities are not available),[2] it is reasonable to offer a shorter period of secondary prophylaxis followed by re-evaluation (including an echocardiogram). This should be done in consultation with the child and family, and with careful consideration of the patient's individual circumstances and family history. In patients with recurrent and/or atypical joint symptoms despite good adherence with penicillin prophylaxis, it is also important to consider the possibility of other rheumatologic diagnoses, such as juvenile idiopathic arthritis.
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]
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