INTRODUCTION
Acute rheumatic fever (ARF) is less common
today than it was 50 years ago. In the late 1940’s
patients with rheumatic fever and rheumatic heart disease
accounted for more than half of schoolchildren recognized
to have cardiovascular problems in the United States. However,
starting in the 1980’s, unexpected scattered outbreaks
of acute rheumatic fever among both adults and children
in North America have confirmed the capacity for this potentially
serious illness to reappear and pose significant public
health problems.7 ARF is a delayed, non-suppurative sequela
of a pharyngeal infection with group A streptococcus. Pharyngitis
usually occurs two to four weeks before the onset of ARF
symptoms. The illness is self-limited but can cause damage
to the heart valves and subsequently lead to cardiac decompensation
and death.3
EPIDEMIOLOGY
Acute rheumatic fever most often occurs
in children, with the peak age of incidence occurring between
5 and 15 years old. Most initial attacks in adults take
place at the end of the second and beginning of the third
decades of life. Although recurrent attacks have occurred
in the fifth and sixth decades of life. Risk factors associated
with individual attacks and outbreaks include a low standard
of living (especially crowding), lower socioeconomic class,
the type of organism and the degree of host immunity to
the prevalent serotypes.5
PATHOGENESIS
The pathogenic mechanisms that lead to
the development of ARF remain incompletely understood. The
pathogenesis has been grouped into three major categories:
(1) a toxic effect of streptococcal extracellular products
on the host tissues, (2) direct infection by the group A
streptococcus and (3) an abnormal or dysfunctional immune
response to one or more as yet unidentified somatic or extracellular
antigens produced by all group A streptococci.5
There is insufficient evidence to support
direct infection of the heart as the inciting event. Also,
while toxins such as streptolysin O and others have been
postulated to be responsible for this sequela, there is
relatively little convincing evidence at the present time.
DIAGNOSIS
The diagnosis of rheumatic fever is a clinical
one, but it does require supporting microbiologic and immunologic
laboratory findings. In 1944, Jones first proposed a set
of criteria to help clinicians appropriately diagnose ARF.
The most recent modification of the Jones Criteria has been
divided into two classes, the “major” and “minor”.
There are five criteria termed major, since they are the
most common ones found in patients with rheumatic fever.
These include carditis, Sydenham’s chorea, migratory
polyarthritis, subcutaneous nodules and erythema margiantum.4
Jones Major Criteria
Carditis is a pancarditis involving the pericardium, myocardium,
epicardium and endocardium. The carditis is characterized
by various signs or symptoms such as sinus tachycardia,
a mitral regurgitation murmur, an S3 gallop, cardiomegaly
and a pericardial friction rub or pleuritic chest pain.3,5
Sydenham’s chorea is a neurologic
disorder consisting of abrupt, purposeless, non-rhythmic
involuntary movements, muscular weakness and emotional disturbances.
Emotional changes can manifest as transient psychosis or
outburst of crying and restlessness. Chorea may have a longer
latent period, up to several months from the onset of streptococcal
infection.5
Migratory polyarthritis usually affects
the ankles, knees, elbows and wrists over a period of days.5
Arthritis (which includes pain and swelling) is usually
the earliest manifestation of ARF. Joint inflammation is
present in several joints, and each joint is inflamed for
no more than one week. Radiographs show a slight effusion
in the affected joint or it may be unremarkable.3 Analysis
of synovial fluid normally reveals a sterile inflammatory
fluid.
Subcutaneous nodules are painless, firm
nodules, of varying size located over bony surfaces or near
tendons. They are present most often in patients with long-standing
rheumatic heart disease.5
Erythema marginatum is an uncommon manifestation
which may present as an evanescent, macular, non-pruritic
pink or faintly red rash that affects the trunk or the limbs,
but not the face. A hot shower may make them more evident.5
Jones Minor Criteria
The minor criteria are nonspecific and include
the following: fever, arthralgias, elevated erythrocyte
sedimentation rate and C-reactive protein and a prolonged
PR interval. The criteria for diagnosis of ARF is either
two major criteria, or one major and two minor criteria,
plus evidence of a previous streptococcal infection.4
The latter may be provided by positive throat culture or
rapid antigen detection test and/or an elevated streptococcal
antibody test.5
COMPLICATIONS
Rheumatic heart disease is a late and most
severe sequela of ARF. It usually occurs 10 to 20 years
after the original attack and is a major cause of acquired
valvular disease in the world. The mitral valve is most
commonly involved, followed by the aortic valve. The classic
finding of rheumatic heart disease is mitral stenosis secondary
to severe calcification, often requiring surgical intervention.6
TREATMENT
There are three goals in the treatment
of ARF: (1) symptomatic relief of the clinical manifestations,
(2) anti-streptococcal therapy and (3) prophylaxis against
future infection to prevent recurrent cardiac disease. The
mainstay for symptomatic relief remains anti-inflammatory
agents, most commonly aspirin. The duration of anti-inflammatory
therapy should be maintained until symptoms are absent and
C-reactive protein (CRP) concentration and erythrocyte sedimentation
rate (ESR) have normalized. The CRP and ESR are both elevated
during the active rheumatic process and are useful lab tests
for monitoring recurrence of inflammation when treatment
is being tapered or has been discontinued.5
Severe carditis can present with congestive
heart failure, cardiomegaly or third-degree heart block.
These patients will need to be treated with conventional
therapy for heart failure. At times, rheumatic carditis
is treated with corticosteroids and aspirin may be added
during a steroid taper.5
Antibiotic therapy with penicillin should
be started immediately for at least 10 days, regardless
if pharyngitis is present or not at time of diagnosis. Individuals
that are allergic to penicillin should be treated with erythromycin
or a narrow spectrum oral cephalosporin.2,5 In addition,
household members and close contacts should have throat
cultures done, and if positive will need to be treated.3
Antibiotic prophylaxis against group A
streptococcal infection is to prevent the recurrence of
ARF. Prophylaxis should be started immediately after resolution
of the acute episode with either parenteral or oral therapy.
Oral agents include penicillin V or sulfadiazine and benzathine
penicillin G as the parenteral regimen. Duration of prophylaxis
is unclear. Some believe it should be continued at least
until the patient is a young adult.1
REFERENCES
- Berrios X, Del Campo E, Guzman B, Bisno AL.
Discontinuing Rheumatic Fever Prophylaxis in Selected Adolescents
and Young Adults. Annals of Internal Medicine 1993;118: 401-406.
- Dajani A, Taubert K, Ferrieri P, Peter G, Shulman
S. Treatment of Acute Streptococcal Pharyngitis
and Prevention of Rheumatic Fever: A statement for Health
Professional. Committee on Rheumatic Fever, Endocarditis,and
Kawasaki Disease of the Council on Cardiovascular Disease
in the Young, the American Heart Association. Pediatrics. 1995;96:758-764.
- Gibofsky A, Zabriskie JB. Clinical Manifestations
and Diagnosis of Acute Rheumatic Fever. Up To Date Online, V.13.2,2005.
- Jones Criteria (revised). For Guidance in the Diagnosis
of Rheumatic Fever. Circulation 1965; 32: 664.
- Kaplan E.L.Rheumatic Fever. Harrison’s Principle
of Internal Medicine. 14th Edition. McGraw Hill, 1998-1309-1311.
- Marcus RH, Sareli, Pocock WA, Barlow JB.
The Spectrum of Severe Rheumatic Valve Disease in a Developing
Country. Annals of Internal Medicine 1994; 120: 177-183.
- Wallace MR, Garst PD, Papadimos TJ, Oldfield
EC. The Return of Acute Rheumatic Fever in Young
Adults. JAMA 1989; 262: 2557.
