Acute Rheumatic Fever Treatment & Management

Updated: Dec 10, 2020
  • Author: Robert J Meador, Jr, MD; Chief Editor: Herbert S Diamond, MD  more...
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Medical Care

Treatment strategies for acute rheumatic fever (ARF) can be divided into the following:

  • Management of the acute attack
  • Management of the current infection
  • Prevention of further infection and attacks

The primary goal of treating an ARF attack is to eradicate streptococcal organisms and bacterial antigens from the pharyngeal region. Penicillin is the drug of choice in persons who are not at risk of allergic reaction. A single parenteral injection of benzathine benzylpenicillin can ensure compliance. Oral cephalosporins, rather than erythromycin, are recommended as an alternative in patients who are allergic to penicillin. However, be cautious of the 20% cross-reactivity of the cephalosporins with penicillin.

Prompt treatment of streptococcal pharyngitis in susceptible hosts can prevent repetitive exposure to pathologically reactive antigens. [26, 27] However, management of the current infection will probably not affect the course of the current attack. Antimicrobial therapy does not alter the course, frequency, or severity of cardiac involvement.

Analgesia is optimally achieved with high doses of salicylates, which often induce dramatic clinical improvement. However, a lower dose may be required to avert symptoms of nausea and vomiting. When salicylates are used as therapy, the dosage should be increased until the drug produces either a clinical effect or systemic toxicity characterized by tinnitus, headache, or hyperpnea.

Corticosteroids should be reserved for the treatment of severe carditis. After 2-3 weeks, the dosage may be tapered, reduced by 25% each week. Overlap with high-dose salicylate therapy is recommended as the dosage of the prednisone is tapered over a 2-week period to avoid poststeroid rebound. In extreme cases, intravenous methylprednisolone may be used.

Mild heart failure usually responds to rest and corticosteroid therapy. Digoxin can be useful in patients with severe carditis, but its use should be monitored closely because of the possibility of heart block.

Nocturnal tachycardia may be a sign of cardiac involvement that may be responsive to digoxin. Vasodilators and diuretics also may be used.

Wilson et al report that treatment with hydroxychloroquine proved effective in 2 patients with rheumatic carditis and a protracted inflammatory course. Hydroxychloroquine has anti-inflammatory and immunomodulatory effects; it suppresses an interleukin-1β–granulocyte-macrophage colony-stimulating factor cytokine axis that is reported to be dysregulated in peripheral blood mononuclear cells of patients with ARF. [28]

Sydenham chorea requires long-term antimicrobial prophylaxis, even if no other manifestations of rheumatic fever evolve. The signs and symptoms of chorea usually do not respond well to treatment with antirheumatic agents. Complete physical and mental rest is essential because the manifestations of chorea may be exaggerated by emotional trauma. Glucocorticoids or salicylates have little or no effect on chorea. Because chorea disappears with sleep, adequate sedation should be provided.

A number of drugs have been used off label for symptomatic treatment of Sydenham chorea, including anticonvulsants (eg, valproate, carbamazepine) and neuroleptics (eg, pimozide, haloperidol, risperidone, olanzapine). Gradual withdrawl of medication is suggested once patients have been symptom free for at least 1 month. [29]

Prevention of ARF has been successful in developed societies. The recommended approach can be divided into primary and secondary prevention. Primary prevention involves eradication of Streptococcus from the pharynx, which generally entails administering a single intramuscular injection of benzathine benzylpenicillin. [30]

For secondary prevention, the American Heart Association (AHA) Committee on Acute Rheumatic Fever recommends a regimen consisting of benzathine benzylpenicillin at 1.2 million units intramuscularly every 4 weeks. However, in high-risk situations, administration every 3 weeks is justified and advised. High-risk situations include patients with heart disease who are at risk of repetitive exposure. [31]

Oral prophylaxis, which is less reliable, consists of phenoxymethylpenicillin (penicillin V) or sulfadiazine. These can be used in compliant patients.

If penicillin allergy is suspected, oral cephalosporins should be used.

Although no consensus on the required duration of antibacterial prophylaxis has been reached, the AHA recommends that prophylaxis be continued for at least 10 years after the last episode of rheumatic fever or until patients are well into adulthood. For those with heart disease who are at risk of repetitive exposures, prophylaxis should be continued for a longer duration, probably indefinitely. However, discontinuing prophylaxis may be reasonable in patients in their third decade of life in whom more than 5 years have passed since their last attack and who are free from rheumatic heart disease.

The principles of treatment include the following:

  • The risk of rheumatic fever recurrence is greatest during the first 3-5 years following the attack.
  • Prophylaxis must continue indefinitely in patients with established heart disease or in those frequently exposed to streptococci.
  • Treatment for an indefinite period is required for patients with frequent exposure to streptococci or for those who are difficult to monitor.

In underdeveloped countries, prophylaxis should be continued as follows:

  • Continue for 5 years after the first attack
  • Continue indefinitely in patients with established heart disease
  • Continue indefinitely in patients who are frequently exposed to streptococci and are difficult to monitor

The decision to withdraw antibacterial treatment should be individualized after carefully assessing the risk of repetitive exposures.


Surgical Care

Valve replacement should be considered in patients with active carditis, especially those with cases that are refractory to medical care or require high doses of vasodilators and diuretics.

Regurgitant lesions respond to valve replacement. Pure stenotic lesions may benefit from more conservative balloon mitral commissurotomy. [32]



Primary care physicians should be considered the patient's advocate and guide to medical resources. The role of specialists is as follows:

  • Rheumatologists usually assist in diagnosis in the face of a substantial differential; when the diagnosis is established, they can advise on the therapy plan

  • A cardiologist should be consulted when cardiac involvement is present

  • A neurologist may offer interventions to help manage chorea



See the list below:

  • All patients should be restricted to bed rest and monitored closely for carditis.

  • Aggressive use of acutely inflamed joints or other exercise may cause permanent joint injury to acutely inflamed joints.

  • When carditis has been documented, a 4-week period of bed rest is recommended. As soon as the signs of acute inflammation subside, patients should resume active ambulation as tolerated.

  • Most patients can be treated safely in an outpatient setting.