Acute Rheumatic Fever Clinical Presentation

  • Author: Robert J Meador, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Sep 8, 2011
 

History

  • Diagnosis is challenging for several reasons, as follows:
    • Approximately 70% of older children and young adults recollect pharyngitis. However, only approximately 20% of young children recollect pharyngitis. Therefore, younger children who present with signs or symptoms consistent with acute rheumatic fever (ARF) merit a higher index of suspicion.
    • The rate of isolation of group A streptococci from the oropharynx is extremely low in all populations.
  • Usually, a latent period of approximately 18 days occurs between the onset of streptococcal pharyngitis and ARF. This latent period is rarely shorter than 1 week or longer than 5 weeks.
    • Typically, the first manifestation is a very painful migratory polyarthritis. Often, associated fever and constitutional toxicity develop.
    • Acute attacks usually resolve within 12 weeks.
  • Guidelines for diagnosis published more than 50 years ago by T. Duckett Jones[3] have been slightly revised by the American Heart Association (AHA).[4] Prior history of a preceding group A streptococcal infection is helpful but not required. In addition, 2 major manifestations or 1 major and 2 minor manifestations must be present.
    • Major manifestations include carditis, polyarthritis, chorea, erythema marginatum, and subcutaneous nodules.
    • Minor manifestations include arthralgias and fever. Laboratory findings include elevated levels of acute-phase reactants (erythrocyte sedimentation rate [ESR] and C-reactive protein) and a prolonged PR interval. A prolonged PR interval is not specific and has not been associated with later cardiac sequelae. The utility of echocardiography is also controversial.
  • The Jones criteria[4] should be viewed as a guide to determine who is at high risk but cannot be used to define diagnosis with absolute certainty.
    • An exception includes chorea, which can present as the sole manifestation of ARF, in spite of negative laboratory results.
    • Another possible exception is indolent carditis.
  • A throat culture with results positive for Streptococcus is found in approximately 25% of patients at the time of presentation.
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Physical

  • Physical findings can be nonspecific and misleading; therefore, a high index of suspicion is required for diagnosis.
  • Suspicious signs for carditis include new or changing valvular murmurs, cardiomegaly, congestive heart failure, and/or pericarditis.
  • Nearly 60% of patients with carditis develop isolated mitral valve involvement, followed in prevalence by combined mitral and aortic valve involvement.
  • When present, Sydenham chorea is seldom evident at the time of initial presentation.
  • Erythema marginatum and subcutaneous nodules are rare (< 10% of patients). See the image below.Erythema marginatum, the characteristic rash of acErythema marginatum, the characteristic rash of acute rheumatic fever.
  • Arthritis, which occurs in 80% of patients, usually involves multiple large joints, particularly the knees, ankles, elbows, and wrists.
    • Hips and smaller joints of hands and feet are less commonly involved.
    • Migratory polyarthritis is usually associated with a febrile illness. It involves a series of painful joints, followed by another series of painful joints.
    • This form of arthritis rarely causes permanent joint deformity.
  • Unusual presentations, such as indolent carditis and isolated chorea, may also occur. Even rarer manifestations include epistaxis and abdominal pain due to serositis.
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Causes

  • Although the mechanism by which streptococcal organisms cause disease is not entirely clear, overwhelming epidemiologic evidence suggests that ARF is caused by streptococcal infection, and recurrences can be prevented with prophylaxis.
  • Strains of group A streptococci that are heavily encapsulated and rich in M protein (signifying virulence in streptococcal strains) seem to be most likely to result in infection.
  • Group A Streptococcus is thought to cause the myriad of clinical diseases in which the host's immunologic response to bacterial antigens cross-react with various target organs in the body, resulting in molecular mimicry. In fact, autoantibodies reactive against the heart have been found in patients with rheumatic carditis. The antibody can cross-react with brain and cardiac antigens, and immune complexes are present in the serum. The problem has been the uncertainty of whether these antibodies are the cause or result of myocardial tissue injury.
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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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