11/1/13 Acute Rheumatic Fever

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Acute Rheumatic Fever
Author: Robert J Meador, MD; Chief Editor: Herbert S Diamond, MD more...

Updated: Sep 8, 2011
Background
The incidence of acute rheumatic fever (ARF) has declined in most developed countries, and many physicians
have little or no practical experience with the diagnosis and management of this condition. Occasional outbreaks
in the United States make complacency a threat to public health.
Diagnosis rests on a combination of clinical manifestations that can develop in relation to group A streptococcal
pharyngitis. These include chorea, carditis, subcutaneous nodules, erythema marginatum, and migratory
polyarthritis. Because the inciting infection is completely treatable, attention has been refocused on prevention.
See the image below.
Clinical manif estations and time course of acute rheumatic f ever.
Pathophysiology
Although the inciting bacterial agent is well known, susceptibility factors remain unclear. The location of the
streptococcal infection seems to play an important role. The clinical syndrome typically follows a streptococcal
pharyngitis, but streptococcal cellulitis has never been implicated.
The earliest and most common feature is a painful migratory arthritis, which is present in approximately 80% of
patients. Large joints such as knees, ankles, elbows, or shoulders are typically affected. Sydenham chorea was
once a common late-onset clinical manifestation but is now rare. Carditis (with progressive congestive heart failure,
a new murmur, or pericarditis) may be the presenting sign of unrecognized past episodes and is the most lethal
manifestation.
Genetics may contribute, as evidenced by an increase in family incidence. No significant association with class-I
human leukocyte antigens (HLAs) has been found, but an increase in class-II HLA antigens DR2 and DR4 has
been found in black and white patients, respectively. Evidence suggests that elevated immune-complex levels in
blood samples from patients with ARF are associated with HLA-B5.
[1]
Epidemiology
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United States
The incidence of an acute rheumatic episode following streptococcal pharyngitis is 0.5-3%. The peak age is 6-20
years. Although the incidence of ARF has steadily declined, the mortality rate has declined even more steeply.
Credit can be attributed to improved sanitation and antibiotic therapy. Several sporadic outbreaks in the United
States could not be blamed directly on poor living conditions. New virulent strains are the best explanation.
International
Most major outbreaks occur under conditions of impoverished overcrowding where access to antibiotics is limited.
Rheumatic heart disease accounts for 25-50% of all cardiac admissions internationally. Regions of major public
health concern include the Middle East, the Indian subcontinent, and some areas of Africa and South America. As
many as 20 million new cases occur each year. The introduction of antibiotics has been associated with a rapid
worldwide decline in the incidence of ARF. Now, the incidence is 0.23-1.88 patients per 100,000 population. From
1862-1962, the incidence declined from 250 patients to 100 patients per 100,000 population, primarily in
teenagers. Notably, natives of Polynesian ancestry in Hawaiian and Maori populations are an exception. The
incidence continues to be 13.4 patients per 100,000 hospitalized children per year.
[2]
Mortality/Morbidity
Mortality rates are steadily improving because of better sanitation and health care.
The current pattern of morbidity is difficult to measure because the first attack of rheumatic fever follows an
unpredictable course. As many as 90% of episodes are clinically contained within 3 months.
Carditis causes the most severe clinical manifestation because heart valves can be permanently damaged.
The disorder also can involve the pericardium, myocardium, and the free borders of valve cusps. Death or
total disability may occur years after the initial presentation of carditis.
Race
An association between certain class-II HLA antigens (DR2 in blacks and DR4 in whites) and ARF has
been reported.
Sex
No general clear-cut sex predilection for the syndrome has been reported, but its manifestations seem to
be sex variable. For example, certain clinical manifestations (ie, chorea and tight mitral stenosis) are
predominant in women, while men are more likely to develop aortic stenosis.
Age
The initial attack of ARF occurs most frequently in persons aged 6-20 years and rarely occurs in persons
older than 30 years.
The disease may cluster in families.
In some countries, a shift into older groups may be a trend.

Contributor Information and Disclosures
Author
Robert J Meador, MD Rheumatology Fellow, Department of Rheumatology, Baylor Garland Family Practice
Clinic
Robert J Meador, MD is a member of the following medical societies: Texas Medical Association
Disclosure: Nothing to disclose.
Coauthor(s)
I Jon Russell, MD, PhD, MS, FACR Director, University Clinical Research Center, Associate Professor,
Department of Internal Medicine, Division of Clinical Immunology and Rheumatology, University of Texas Health
Science Center at San Antonio
I Jon Russell, MD, PhD, MS, FACR is a member of the following medical societies: Alpha Omega Alpha,
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American College of Physicians, American College of Rheumatology, International Association for the Study of
Pain, and International MYOPAIN Society (IMS)
Disclosure: Jazz Pharma Consulting fee Consulting; Pfizer Pharma Grant/research funds Independent
contractor; Pfizer Pharma Consulting fee Speaking and teaching; Lily Pharma Grant/research funds
Independent contractor; Lily Pharma Consulting fee Speaking and teaching
Specialty Editor Board
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center
College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson
University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical
Center
Lawrence H Brent, MD is a member of the following medical societies: American Association for the
Advancement of Science, American Association of Immunologists, American College of Physicians, and
American College of Rheumatology
Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech
Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting;
Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and
teaching; UCB Honoraria Speaking and teaching
Alex J Mechaber, MD, FACP Senior Associate Dean for Undergraduate Medical Education, Associate
Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American
College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.
Chief Editor
Herbert S Diamond, MD Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh
School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College
of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest
Other
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