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Treatment
The sine qua non of treatment is acute antimicrobial therapy to eliminate GABHS from the pharynx and subsequent continuous antibiotics for secondary prevention (Table 88- !" #rimary prevention$ %ith effective antibiotic treatment startin& less than '( days after the onset of pharyn&itis$ lar&ely eliminates ris) of *+ and is the most cost-effective approach" ,nce the *+ is manifested$ the treatment al&orithm varies$ dependin& on manifestations of ma-or criteria (+i&" 88-.!" The course of *+ covers a spectrum from mild$ resolvin& %ithout treatment$ to severe and recurrent %ith consequent end-sta&e *H/" 0on&-term monitorin& is %arranted$ even if symptoms resolve early$ because approximately half of carditis patients develop *H/" 12$''3 The first line of symptomatic therapy has traditionally been anti-inflammatory a&ents$ ran&in& from salicylates to steroids$ althou&h the course of the disease is not influenced by anti-inflammatory therapy" 1 (3 4vidence for bed rest is from the preantibiotic era$ and many practitioners no% treat *+ patients on an outpatient basis$ except for those presentin& %ith carditis$ for %hom bed rest$ at a minimum durin& the symptomatic sta&e$ is empirically applied" TABLE 88-2 -- Antibiotic Therapy for Acute Rheumatic Fever and Long-Term Prophyla i! "nitial Treatment of #roup A Beta-$emolytic %treptococcal Pharyngiti! &Adult 'o!age!( A)T"B"*T"+ Ben5athine penicillin G #enicillin ; Amoxicillin Penicillin Allergic =arro%-spectrum cephalosporins> ;aries by dru& .(( m& #, <(( m& #, day ' <( m& #, days -< <( m& #, ;aries by dru& Avoid if history of anaphylaxis secondary to penicillin '*%E FRE,-E)+. '-RAT"*) +*//E)T% Acutely only '( days '( days 8 9ompliance issues : #ain +LA%% 6

'" million ,ne time units 67 <(( m& #, '((( m& #, T%ice daily /aily

6 6

'( days

9lindamycin> $ 1?3

T%ice daily

'( days

66a

A5ithromycin> $ 1@3

/aily

< days

66a

9larithromycin> $ 1@3

T%ice daily

'( days

66a

%econdary Prophyla i! Regimen for Patient! 0ith 'ocumented RF &Adult 'o!age!( 123 A)T"B"*T"+ Ben5athine penicillin G #enicillin ; 4rythromycin> $ Sulfadia5ine> Sulfisoxa5ole>
1@3

'*%E '" million units 67 <( m& #, <( m& #, ' & #, ' & #,

FRE,-E)+. 4very . to 2 %ee)s 1A3 T%ice daily T%ice daily /aily /aily

+*//E)T% 8 9ompliance issues : #ain

+LA%% 6 6 6 6 66a

Modified from Gerber MA, Baltimore RS, Eaton CB, et al: Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal phar ngitis! A scientific statement from the American "eart Association

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Rheumatic #ever, Endocarditis, and $a%asa&i 'isease Committee! Circulation (():(*+(, ,--). and Rheumatic fever and rheumatic heart disease! /orld "ealth 0rgan 1ech Rep Ser ),2:(, ,--+!
*

Alternative for penicillin-allergic patients. Erythromycin for secondary prophylaxis is an alternative for patients allergic to both penicillin and sulfa. Dosage is empiric. For severe pharyngitis, doses up to 1. g daily in t!o to four divided doses. "ome areas have a high rate of macrolide-resistant group A streptococci. #n addition, erythromycin toxicity, including gastrointestinal intolerance and long-$% syndrome, limits its use. Duration of therapy ranges from & years to life-long 'see text(. For patients !ith poststreptococcal reactive arthritis, recommended duration is 1 year in nonendemic areas, & years !here )F is prevalent if no evidence of carditis appears. #n endemic areas, ben*athine penicillin every + !ee,s should be considered to maintain optimal drug levels '-lass # indication(. #n nonendemic areas, ben*athine penicillin is given every + !ee,s if )F recurs on the regimen of every . !ee,s '-lass #(.

FIGURE 88-3 Algorithm for management of rheumatic fever and its primary manifestations. /"alicylates are also indicated for fever and arthralgia, but there is no evidence of effectiveness for carditis or chorea. P")A 0 poststreptococcal reactive arthritis1 )F 0 rheumatic fever.3Modified from 1hatai ', 1uri 4G: Current guidelines for the treatment of patients %ith rheumatic fever! 'rugs *5:*+*, ()))!6

Prevention Strategies Primary Prevention

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Effective eradication of GABHS from the pharynx defines the role of primary prevention. Patients with apparent bacterial pharyngitis and positive test results for GABHS should be treated as early as possible in the suppurative phase. he differential diagnosis! in addition to viral infection! includes non"GABHS and gonococcal pharyngitis. Penicillin is uniformly effective for GABHS if it is ta#en orally for a full $%"day course or if intramuscular ben&athine penicillin is administered because penicillin"resistant GABHS has not been demonstrated. he particular advantage of intramuscular ben&athine penicillin G is that it avoids compliance issues. 'ral cephalosporins! indicated for penicillin"allergic patients! have been used in shorter than $%"day courses with high compliance! with bacterial elimination and clinical response that may be superior to penicillin treatment! but the evidence base is insufficient to recommend this regimen for treatment in endemic areas. Aggressive antibiotic therapy for primary prevention is essential in areas where () is prevalent and may represent the best hope for decreasing the overall health care burden of (H*. +,$.n contrast! in populations where () is rare! antibiotic use results in modest therapeutic benefit! and the ris#"to"benefit ratio has been called into /uestion.

Secondary Prevention
he method of choice for prevention of () recurrence is continuous administration of ben&athine penicillin G every 0 wee#s. Because of low penicillin levels during the fourth wee#! 1"wee# intervals should be considered in endemic areas or for patients at high ris#. hose with documented () should have continuous secondary prevention as soon as the primary GABHS treatment regimen has been completed. An oral regimen should be reserved primarily for patients deemed to be at low ris# for recurrent () or those allergic to penicillin or otherwise intolerant of intramuscular therapy. he duration of therapy depends on the patient2s age! #nown (H*! time since last episode of ()! number of episodes! family history! occupational exposure! and environmental factors! such as living in endemic areas. +1- he 3lass . recommendations are 4 years or until the age of ,$ years 5whichever is longer6 in the absence of carditis! $% years or until the age of ,$ years for patients with mild or apparently healed carditis! and $% years or until the age of 0% years for patients who develop (H*. +,- Patients at high ris# for repeated episodes of ()! such as those at significant ris# of recurrent exposure to GABHS infection! should be considered for life"long antibiotic prophylaxis. 3onfounding factors have been reluctance of rural practitioners to administer parenteral antibiotics for fear of allergic reactions and! for similar reasons! regulations prohibiting parenteral administration in hospitals in some developing countries. he actual ris# of anaphylaxis! estimated to be %.,7! is less in children younger than $, years.

Therapeutic 3arditis

o!a"ities

Salic lates and nonsteroidals have no specific role in rheumatic carditis, %ith the e7ception of treatment of concomitant pericarditis! Acute carditis has generall been treated %ith steroids even though a meta8anal sis of eight randomi9ed controlled trials failed to demonstrate superiorit of steroids, immunoglobulins, or salic lates over placebo in the progression of R"'! :,-; <evertheless, in the setting of severe, potentiall life8 threatening heart failure, steroid administration is %idespread! /ithdra%al of steroids or salic lates can result in rebound or relapse! 1reatment of cardiac manifestations other%ise follo%s established guidelines, including management of congestive heart failure and severe valvular regurgitation, although special attention should be paid to use of digitalis because these patients are sensitive to development of heart bloc&!

=nless valvular regurgitation and severe congestive heart failure are refractor to drug therap , valve surger is avoided %henever possible during R#! Surgical morbidit and mortalit have been significant, and failed repair leading to valve replacement has been fre>uent! "o%ever, %hen surger is necessar , ?@ function generall improves significantl , consistent %ith valve regurgitation rather than m ocardial d sfunction being the primar mechanism leading to heart failure!

Arthritis

Salic lates are the first line of therap for migrator pol arthritis because of their highl effective analgesic, anti8inflammator , and antip retic properties! <onsteroidals are

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effective alternatives! Aspirin 3up to (-- mgA&gAda in four or more divided doses6 is both therapeutic and diagnostic. failure of the pain to resolve %ithin ,+ hours suggests alternative causes of arthritis! Although salic late levels can be follo%ed 3(* to 2- mgAd? is the therapeutic range6, these data are usuall not available in endemic areas. instead, patients are monitored for tinnitus and gastrointestinal to7icit ! Earl administration does have the potential to mas& the evolving clinical picture 3e!g!, arthritis %hen medication is given for arthralgias6! Steroids are t picall not used because the offer no therapeutic advantage and ma mas& the presence of other illnesses causing arthritis, such as lupus, or e7acerbate other causes, such as infectious arthritis!

3horea
1raditional treatment has included sedation and empiric use of antisei9ure or antips chotic medications! Small series have studied corticosteroids, along %ith plasmapheresis and intravenous immunoglobulins, to assess their influence on the severit and time course of s mptoms! 1he duration of s mptoms does appear to be shortened %ith treatment! "o%ever, the evidence base is not conclusive, some of the interventions are potentiall to7ic, and, pending larger studies, a conservative approach to a largel self8limited disorder has been deemed appropriate! #or patients %ith refractor s mptoms, there is modest evidence favoring use of carbama9epine or valproic acid! Because of the high incidence of carditis, :(B; %hether clinical or subclinical, and potential progression to R"', these patients re>uire long8term antibiotic therap !

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