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Pediatric Rheumatic Fever Treatment & Management

  • Author: Thomas K Chin, MD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Jun 28, 2016
 

Medical Care

Prevention of rheumatic fever in patients with group A beta hemolytic streptococci (GABHS) pharyngitis

For patients with GABHS pharyngitis, a meta-analysis supported a protective effect against rheumatic fever (RF) when penicillin is used following the diagnosis.[18]

  • Oral (PO) penicillin V remains the drug of choice for treatment of GABHS pharyngitis, but ampicillin and amoxicillin are equally effective.
  • When PO penicillin is not feasible or dependable, a single dose of intramuscular benzathine penicillin G, or benzathine/procaine penicillin combination is therapeutic.
  • For patients who are allergic to penicillin, administer erythromycin or a first-generation cephalosporin. Other options include clarithromycin for 10 days, azithromycin for 5 days, or a narrow-spectrum (first-generation) cephalosporin for 10 days. As many as 15% of penicillin-allergic patients are also allergic to cephalosporins.
  • Do not use tetracyclines and sulfonamides to treat GABHS pharyngitis.
  • For recurrent group A streptococci (GAS) pharyngitis, a second 10-day course of the same antibiotic may be repeated. Alternate drugs include narrow-spectrum cephalosporins, amoxicillin-clavulanate, dicloxacillin, erythromycin, or other macrolides.
  • Control measures for patients with GABHS pharyngitis are as follows:
    • Hospitalized patients: Place hospitalized patients with GABHS pharyngitis of pneumonia on droplet precautions, as well as standard precautions, until 24 hours after initiation of appropriate antibiotics.
    • Exposed persons: People in contact with patients having documented cases of streptococcal infection first should undergo appropriate laboratory testing if they have clinical evidence of GABHS infection and should undergo antibiotic therapy if infected.
    • School and childcare centers: Children with GABHS infection should not attend school or childcare centers for the first 24 hours after initiating antimicrobial therapy.
  • GABHS carriage is difficult to eradicate with conventional penicillin therapy. Thus, PO clindamycin (20 mg/kg/d PO in 3 divided doses for 10 d) is recommended.
  • In general, antimicrobial therapy is not indicated for pharyngeal carriers of GABHS. Exceptions include the following:
    • Outbreaks of rheumatic fever or poststreptococcal glomerulonephritis
    • Family history of rheumatic fever
    • During outbreaks of GAS pharyngitis in a closed community
    • When tonsillectomy is considered for chronic GABHS carriage
    • When multiple episodes of documented GABHS pharyngitis occur within a family despite appropriate therapy
    • Following GAS toxic shock syndrome or necrotizing fasciitis in a household contact

Treatment for patients with rheumatic fever

Therapy is directed towards eliminating the GABHS pharyngitis (if still present), suppressing inflammation from the autoimmune response, and providing supportive treatment of congestive heart failure (CHF).

  • Treat residual GABHS pharyngitis as outlined above, if still present.
  • Treatment of the acute inflammatory manifestations of acute rheumatic fever consists of salicylates and steroids. Aspirin in anti-inflammatory doses effectively reduces all manifestations of the disease except chorea, and the response typically is dramatic.
    • If rapid improvement is not observed after 24-36 hours of therapy, question the diagnosis of rheumatic fever.
    • Attempt to obtain aspirin blood levels from 20-25 mg/dL, but stable levels may be difficult to achieve during the inflammatory phase because of variable GI absorption of the drug. Maintain aspirin at anti-inflammatory doses until the signs and symptoms of acute rheumatic fever are resolved or residing (6-8 wk) and the acute phase reactants (APRs) have returned to normal.
    • Anti-inflammatory doses of aspirin may be associated with abnormal liver function tests and GI toxicity, and adjusting the aspirin dosage may be necessary.
    • When discontinuing therapy, withdraw aspirin gradually over weeks while monitoring the APRs for evidence of rebound. Chorea most frequently is self-limited but may be alleviated or partially controlled with phenobarbital or diazepam.
  • If moderate to severe carditis is present as indicated by cardiomegaly, third-degree heart block, or CHF, add PO prednisone to salicylate therapy.
    • Continue prednisone for 2-6 weeks depending on the severity of the carditis, and taper prednisone during the last week of therapy.
    • Discontinuing prednisone therapy after 2-4 weeks, while maintaining salicylates for an additional 2-4 weeks, can minimize adverse effects.
  • Include digoxin and diuretics, afterload reduction, supplemental oxygen, bed rest, and sodium and fluid restriction as additional treatment for patients with acute rheumatic fever and CHF. The diuretics most commonly used in conjunction with digoxin for children with CHF include furosemide and spironolactone.
    • Initiate digoxin only after checking electrolytes and correcting abnormalities in serum potassium.
    • The total loading dose is 20-30 mcg/kg PO every day, with 50% of the dose administered initially, followed by 25% of the dose 8 hours and 16 hours after the initial dose. Maintenance doses typically are 8-10 mcg/kg/d PO in 2 divided doses. For older children and adults, the total loading dose is 1.25-1.5 mg PO, and the maintenance dose is 0.25-0.5 mg PO every day. Therapeutic digoxin levels are present at trough levels of 1.5-2 ng/mL.
  • Afterload reduction (ie, using ACE inhibitor captopril) may be effective in improving cardiac output, particularly in the presence of mitral and aortic insufficiency. Start these agents judiciously. Use a small, initial test dose (some patients have an abnormally large response to these agents), and administer only after correcting hypovolemia.
  • When heart failure persists or worsens during the acute phase after aggressive medical therapy, surgery is indicated to decrease valve insufficiency.

Treatment for patients following rheumatic heart disease (RHD)

Preventive and prophylactic therapy is indicated after rheumatic fever and RHD to prevent further damage to valves.

  • Primary prophylaxis (initial course of antibiotics administered to eradicate the streptococcal infection) also serves as the first course of secondary prophylaxis (prevention of recurrent rheumatic fever and RHD).
  • An injection of 0.6-1.2 million units of benzathine penicillin G intramuscularly every 4 weeks is the recommended regimen for secondary prophylaxis for most US patients. Administer the same dosage every 3 weeks in areas where rheumatic fever is endemic, in patients with residual carditis, and in high-risk patients.
    • Although PO penicillin prophylaxis is also effective, data from the World Health Organization indicate that the recurrence risk of GABHS pharyngitis is lower when penicillin is administered parentally.
    • The duration of antibiotic prophylaxis is controversial. Continue antibiotic prophylaxis indefinitely for patients at high risk (eg, health care workers, teachers, daycare workers) for recurrent GABHS infection. Ideally, continue prophylaxis indefinitely, because recurrent GABHS infection and rheumatic fever can occur at any age; however, the American Heart Association currently recommends that patients with rheumatic fever without carditis receive prophylactic antibiotics for 5 years or until aged 21 years, whichever is longer.[19] Patients with rheumatic fever with carditis but no valve disease should receive prophylactic antibiotics for 10 years or well into adulthood, whichever is longer. Finally, patients with rheumatic fever with carditis and valve disease should receive antibiotics at least 10 years or until aged 40 years.
    • Patients with RHD and valve damage require a single dose of antibiotics 1 hour before surgical and dental procedures to help prevent bacterial endocarditis. Patients who had rheumatic fever without valve damage do not need endocarditis prophylaxis. Do not use penicillin, ampicillin, or amoxicillin for endocarditis prophylaxis in patients already receiving penicillin for secondary rheumatic fever prophylaxis (relative resistance of PO streptococci to penicillin and aminopenicillins). Alternate drugs recommended by the American Heart Association for these patients include PO clindamycin (20 mg/kg in children, 600 mg in adults) and PO azithromycin or clarithromycin (15 mg/kg in children, 500 mg in adults). Additional guidelines for endocarditis prophylaxis in patients who are allergic to penicillin or who are unable to receive PO antibiotics are discussed in the Bacterial Endocarditis article.
    • A recent study investigated the difference in clinical manifestations and outcomes between first episode and recurrent rheumatic fever.[20] The study concluded that subclinical carditis occurred only in patients experiencing the first episode, and that all deaths occurred in patients with recurrent rheumatic fever, emphasizing the need for secondary prophylaxis.
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Surgical Care

When heart failure persists or worsens after aggressive medical therapy for acute RHD, surgery to decrease valve insufficiency may be lifesaving. Approximately 40% of patients with acute rheumatic fever subsequently develop mitral stenosis as adults. Mitral valvulotomy, percutaneous balloon valvuloplasty, or mitral valve replacement may be indicated in patients with critical stenosis. Traditionally, valve replacement appears to be the preferred surgical option for patients with high rates of recurrent symptoms after annuloplasty or other repair procedures. However, recent modifications of standard repair techniques, adherence to the importance of good leaflet coaptation, and strict quality control with stringent use of intraoperative transesophageal echocardiography have all contributed to the improved long-term results.[21]

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Diet

Advise nutritious diet without restrictions except in patients with CHF, who should follow a fluid-restricted and sodium-restricted diet. Potassium supplementation may be necessary because of the mineralocorticoid effect of corticosteroid and the diuretics, if used.

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Activity

Initially, place patients on bed rest, followed by a period of indoor activity before they are permitted to return to school. Do not allow full activity until the APRs have returned to normal. Patients with chorea may require a wheelchair and should be on homebound instruction until the abnormal movements resolve.

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Contributor Information and Disclosures
Author

Thomas K Chin, MD Professor of Pediatrics, Chief of Pediatric Cardiology, Pennsylvania State University College of Medicine

Thomas K Chin, MD is a member of the following medical societies: American Academy of Pediatrics, American Heart Association, American College of Cardiology

Disclosure: Nothing to disclose.

Coauthor(s)

Douglas Li, MD Assistant Clinical Professor, Division of Pediatric Pulmonology, Department of Pediatrics, University of California, Los Angeles, David Geffen School of Medicine, Mattel Children's Hospital UCLA

Douglas Li, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Thomas JA Lehman, MD FAAP, FACR, Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill Cornell Medical College; Chief, Hospital for Special Surgery

Thomas JA Lehman, MD is a member of the following medical societies: PM American Allergy Society

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

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Clinical manifestations and time course of acute rheumatic fever.
Chest radiograph showing cardiomegaly due to carditis of acute rheumatic fever.
Erythema marginatum, the characteristic rash of acute rheumatic fever.
 
 
 
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