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 manifestations and time course of acute rClinical manifestations and time course of acute rheumatic fever.
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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]

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Epidemiology

Frequency

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.
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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, 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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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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