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AHA Guidelines on Prevention of Rheumatic Fever and Diagnosisand Treatment of Acute Streptococcal PharyngitisCARRIE ARMSTRONG

Am Fam Physician. 2010 Feb 1;81(3):346­359.

Guideline source: American Heart Association

Literature search described? No

Evidence rating system used? Yes

Published source: Circulation, March 24, 2009

Available at: http://circ.ahajournals.org/content/vol119/issue11 (http://circ.ahajournals.org/content/vol119/issue11)

Although the overall incidence of acute rheumatic fever and rheumatic heart disease is low in most areas of the United States, they are the leading causes ofcardiovascular death during the first five decades of life in developing countries. This disparity serves as a reminder of the importance of continued vigilance toprevent these diseases. The American Heart Association (AHA) recently updated its recommendations on the prevention of rheumatic fever.

Primary Prevention of Rheumatic FeverGroup A streptococcus (GAS) infections of the pharynx are the precipitating cause of rheumatic fever. Proper diagnosis and adequate antibiotic treatment of GASinfections can prevent acute rheumatic fever in most cases.

DIAGNOSIS OF STREPTOCOCCAL PHARYNGITISAcute pharyngitis is caused much more often by viruses than by bacteria. However, differentiation of GAS pharyngitis from other causes of acute pharyngitis isoften difficult because none of the clinical findings suggestive of GAS infection is specific enough on its own for diagnosis (Table 1). A history of recent exposure ishelpful in making the diagnosis, as is an awareness of the prevalence of GAS infections in the community.

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Tale 1.

GAS vs. Viral Pharyngitis: Clinical and Epidemiologic Findings

Features suggestive of GAS infection

Beefy, swollen, red uvula

Fever

Headache

History of exposure to GAS

Nausea, vomiting, and abdominal pain

Pain with swallowing

Patient 5 to 15 years of age

Presentation in winter or early spring (in temperate climates)

Scarlet fever rash

Soft palate petechiae (“doughnut lesions”)

Sudden onset of sore throat

Tender, enlarged anterior cervical nodes

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If clinical and epidemiologic findings suggest GAS infection, microbiologic confirmation with a throat culture or rapid antigen detection test (RADT) is required. Thediagnosis of GAS pharyngitis is more easily excluded than confirmed, so testing usually is unnecessary in patients with findings suggestive of a viral origin.Treatment is indicated for patients with acute pharyngitis who have a positive throat culture or RADT. However, because of the low sensitivity of many RADTs, anegative test does not exclude GAS infection, and a throat culture usually should be performed. The exception is in adults, in whom the incidence of GASpharyngitis and the risk of acute rheumatic fever are low. In this population, diagnosis of GAS pharyngitis can be made on the basis of an RADT alone, withoutconfirmation of negative results by a throat culture.

Antistreptococcal antibody titers reflect past—not present—immunologic events and therefore cannot be used to determine whether a patient with pharyngitis andGAS in the pharynx is truly infected or merely a streptococcal carrier. When present, elevated or increasing antistreptococcal titers can confirm a recent GASinfection and are valuable in identifying a preceding GAS infection in a patient suspected of having rheumatic fever.

TREATMENT OF STREPTOCOCCAL PHARYNGITISPrimary prevention of rheumatic fever requires adequate therapy for GAS pharyngitis. In selecting a treatment regimen, physicians should consider bacteriologicand clinical effectiveness, ease of adherence to the recommended regimen (i.e., dosing frequency, duration of therapy, and palatability), cost, spectrum of activityof the selected agent, and potential adverse effects.

Intramuscular penicillin G benzathine, oral penicillin V potassium, and oral amoxicillin are the recommended antimicrobial agents for the treatment of GASpharyngitis in persons without penicillin allergy (Table 2). GAS resistance to penicillin has never been documented, and penicillin prevents primary attacks ofrheumatic fever even when started nine days after illness onset. Patients are no longer considered contagious after 24 hours of antibiotic therapy.

Penicillin V potassium is preferred over penicillin G benzathine because it is more resistant to gastric acid. However, penicillin G benzathine should be considered inpatients who are unlikely to complete a 10­day course of oral therapy, in those with personal or family histories of rheumatic fever or rheumatic heart failure, and inthose with environmental factors that put them at risk for rheumatic fever (e.g., crowded living conditions, low socioeconomic status).

OTHER RECOMMENDATIONSBecause most patients with GAS pharyngitis respond well to antimicrobial therapy, posttreatment throat cultures are indicated only in those who remainsymptomatic, who have recurrent symptoms, or who have had rheumatic fever previously.

With the exception of persons who have had or whose family members have had rheumatic fever, repeated courses of antibiotics are typically not indicated inasymptomatic persons who continue to harbor GAS after appropriate therapy.

Although acute infections with group B and C beta­hemolytic streptococci can appear similar to GAS pharyngitis, rheumatic fever has not been documented as acomplication of these infections.

Secondary Prevention of Rheumatic FeverRecurrent rheumatic fever is associated with worsening or development of rheumatic heart disease. Prevention of recurrent GAS pharyngitis is the most effectivemethod of preventing severe rheumatic heart disease. However, a GAS infection does not have to be symptomatic to trigger a recurrence, and rheumatic fever canrecur even when a symptomatic infection is treated optimally. Therefore, prevention of recurrent rheumatic fever requires continuous antimicrobial prophylaxisrather than recognition and treatment of acute episodes of GAS pharyngitis.

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Tale 2.

Primary Prevention of Rheumatic Fever

AGENT DOSAGE EVIDENCERATING*

Penicillins

Amoxicillin 50 mg per kg (maximum, 1 g) orally once daily for 10 days 1B

Penicillin G benzathine Patients weighing 27 kg (60 lb) or less: 600,000 units IM once 1B

Patients weighing more than 27 kg: 1,200,000 units IM once

Penicillin V potassium Patients weighing 27 kg or less: 250 mg orally 2 or 3 times daily for 10 days 1B

Patients weighing more than 27 kg: 500 mg orally 2 or 3 times daily for 10 days

For patients allergic to penicillin

Narrow­spectrum cephalosporin (cephalexin [Keflex], cefadroxil[formerly Duricef])†

Varies 1B

Azithromycin (Zithromax) 12 mg per kg (maximum, 500 mg) orally once daily for 5 days 2aB

Clarithromycin (Biaxin)‡ 15 mg per kg orally per day, divided into 2 doses (maximum, 250 mg twicedaily), for 10 days

2aB

Clindamycin (Cleocin) 20 mg per kg orally per day (maximum, 1.8 g per day), divided into 3 doses, for10 days

2aB

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SECONDARY PROPHYLAXISContinuous prophylaxis is recommended in patients with well­documented histories of rheumatic fever and in those with evidence of rheumatic heart disease(Tables 3 and 4). Prophylaxis should be initiated as soon as acute rheumatic fever or rheumatic heart disease is diagnosed. To eradicate residual GAS, a fullcourse of penicillin should be given to patients with acute rheumatic fever, even if a throat culture is negative.

Continuous antimicrobial prophylaxis provides the most effective protection from recurrences of rheumatic fever. Because the risk of recurrence depends on manyfactors, physicians should determine the appropriate duration of prophylaxis on a case­by­case basis while also considering the presence of rheumatic heartdisease. Patients who have had rheumatic carditis, with or without valvular disease, are at high risk of recurrences and are likely to have increasingly severe cardiacinvolvement with each episode. These patients should receive long­term antibiotic prophylaxis well into adulthood, and perhaps for life. Patients with persistentvalvular disease should receive prophylaxis for 10 years after the last episode of acute rheumatic fever or until 40 years of age, whichever is longer. At that time,the severity of valvular disease and the potential for exposure to GAS should be determined, and continued prophylaxis (possibly lifelong) should be considered inhigh­risk patients.

In the United States, an injection of penicillin G benzathine every four weeks is the recommended prophylactic regimen for secondary prevention in mostcircumstances. In certain populations, administration every three weeks is justified because serum drug levels may fall below a protective level before four weeksafter the initial dose. A three­week dosing regimen is recommended only for patients who have recurrent acute rheumatic fever despite adherence to a four­week

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Tale 3.

Duration of Secondary Prophylaxis for Rheumatic Fever

TYPE DURATION AFTER LAST ATTACK EVIDENCERATING*

Rheumatic fever with carditis and residual heart disease (persistentvalvular disease†)

10 years or until age 40 years (whichever is longer); lifetime prophylaxismay be needed

1C

Rheumatic fever with carditis but no residual heart disease (no valvulardisease†)

10 years or until age 21 years (whichever is longer) 1C

Rheumatic fever without carditis 5 years or until age 21 years (whichever is longer) 1C

*—American Heart Association evidence ratings: 1C = case studies, standard of care, or consensus opinion that a procedure or treatment is beneficial, useful, and effective.

†—Clinical or echocardiographic evidence.

Adapted from Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from theAmerican Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council onFunctional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics.Circulation. 2009;119(11):1547.

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Tale 4.

Secondary Prevention of Rheumatic Fever

AGENT DOSAGE EVIDENCERATING*

Penicillin G benzathine Patients weighing 27 kg (60 lb) or less: 600,000 units IM every 4weeks†

1A

Patients weighing more than 27 kg: 1,200,000 units IM every 4weeks†

Penicillin V potassium 250 mg orally twice daily 1B

Sulfadiazine Patients weighing 27 kg or less: 0.5 g orally once daily 1B

Patients weighing more than 27 kg: 1 g orally once daily

Macrolide or azalide antibiotic (for patients allergic to penicillin andsulfadiazine)‡

Varies 1C

IM = intramuscularly.

*—American Heart Association evidence ratings: 1A = evidence from multiple randomized trials or meta­analyses that a procedure or treatment is beneficial, useful, and effective; 1B =evidence from a single randomized trial or nonrandomized studies that a procedure or treatment is beneficial, useful, and effective; 1C = case studies, standard of care, or consensusopinion that a procedure or treatment is beneficial, useful, and effective.

†—Administration every 3 weeks is recommended in certain high­risk situations.

‡—Macrolide antibiotics should not be used in persons taking other medications that inhibit cytochrome P450 3A, such as azole antifungal agents, human immunodeficiency virusprotease inhibitors, and some selective serotonin reuptake inhibitors.

Adapted from Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the

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regimen. The advantages of penicillin G benzathine should be weighed against the inconvenience to the patient and the pain of injection, which causes somepatients to discontinue prophylaxis.

Successful oral prophylaxis depends on patient adherence to the prescribed regimen. Patients should be given careful, repeated instructions about the importanceof compliance to the dosing regimen. Even with optimal patient compliance, the risk of recurrence is higher in patients receiving oral prophylaxis than in thosereceiving injections of penicillin G benzathine. Therefore, oral regimens are more appropriate for patients at lower risk of recurrent rheumatic fever.

Bacterial EndocarditisThe AHA no longer recommends prophylaxis for infective endocarditis in most patients with rheumatic heart disease. The exceptions are patients with prostheticvalves or valves repaired with prosthetic material, patients with previous endocarditis or specific forms of congenital heart disease, and cardiac transplant recipientswho develop cardiac valvulopathy. In these patients, an agent other than penicillin should be used to prevent infective endocarditis, because alpha­hemolyticstreptococci have likely developed resistance to penicillin.

Poststreptococcal Reactive ArthritisPoststreptococcal reactive arthritis (PSRA) may occur after an episode of GAS pharyngitis in patients who do not have any other major criteria of acute rheumaticfever. PSRA generally follows a symptom­free interval of about 10 days after the GAS pharyngitis, is cumulative and persistent, involves the large and small jointsand the axial skeleton, and does not respond to aspirin therapy. In contrast, arthritis associated with rheumatic fever occurs two to three weeks after an episode ofGAS pharyngitis, is migratory and transient, involves only the large joints, and responds rapidly to aspirin therapy.

Although all patients with PSRA have serologic evidence of a recent GAS infection, GAS is isolated in no more than one half of these patients who have a throatculture. Because valvular heart disease can develop in patients with PSRA, secondary prophylaxis should be administered for up to one year after symptom onset,and these patients should be observed for several months for clinical evidence of carditis. If such evidence is not observed, prophylaxis can be discontinued.However, if valvular disease is detected, the patient should be classified as having had acute rheumatic fever, and secondary prophylaxis should be continued.

PANDASIt has been proposed that an autoimmune response after a streptococcal infection may result in obsessive­compulsive disorder or tics in some children. Thisconcept, known as PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections), is controversial, and the current evidencesuggests that it should be considered a yet­unproven hypothesis. Until a causal relationship has been established between PANDAS and GAS infections, routinelaboratory testing for GAS is not recommended to diagnose this disorder, and long­term prophylaxis or immunoregulatory therapy is not recommended.

Coverage of guidelines from other sources does not imply endorsement by AFP or the AAFP.

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