Rheumatic Fever in Emergency Medicine Clinical Presentation

  • Author: Steven J Parrillo, DO, FACOEP, FACEP; Chief Editor: Robert E O'Connor, MD, MPH   more...
 
Updated: Mar 22, 2012
 

History

Acute rheumatic fever (ARF) is associated with 2 distinct patterns of presentation. The first pattern of presentation is sudden onset. It typically begins as polyarthritis 2-6 weeks after streptococcal pharyngitis and is usually characterized by fever and toxicity. If the initial abnormality is mild carditis, ARF may be insidious or subclinical.

Age at onset influences the order of complications. Younger children tend to develop carditis first, whereas older patients tend to develop arthritis first.

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Physical

Diagnosis of acute rheumatic fever (ARF) requires a high index of suspicion.

Guidelines of diagnosis used by the American Heart Association include major and minor criteria (ie, modified Jones criteria). In addition to evidence of a previous streptococcal infection, the diagnosis requires 2 major Jones criteria or 1 major plus 2 minor Jones criteria.

Major criteria are as follows:

  • Carditis: This occurs in as many as 40% of patients and may include cardiomegaly, new murmur, congestive heart failure, and pericarditis, with or without a rub and valvular disease.
  • Migratory polyarthritis: This condition occurs in 75% of patients and is polyarticular, fleeting, and involves the large joints. Note that one group of authors has suggested that atypical cases may only involve small joints.[12]
  • Subcutaneous nodules (ie, Aschoff bodies): These nodules occur in 10% of patients and are edematous, fragmented collagen fibers. They are firm, painless nodules on the extensor surfaces of the wrists, elbows, and knees.
  • Erythema marginatum: This condition occurs in about 5% of patients. The rash is serpiginous and long lasting.
  • Chorea (also known as Sydenham chorea and "St Vitus dance"): This characteristic movement disorder occurs in 5-10% of cases. Sydenham chorea consists of rapid, purposeless movements of the face and upper extremities. Onset may be delayed for several months and may cease when the patient is asleep. Note that a murmur in a patient with Sydenham's predicts carditis with a high degree of probability.[24]

Minor criteria are as follows:

  • Clinical findings include arthralgia, fever, and previous history of ARF.
  • Laboratory findings include elevated acute-phase reactants (eg, erythrocyte sedimentation rate, C reactive protein), a prolonged PR interval, and supporting evidence of antecedent group A streptococcal infections (ie, positive throat culture or rapid streptococcal screen and an elevated or rising streptococcal antibody titer).

Numerous authors have suggested that changes to the Jones Criteria may be in order. For example, some have suggested that echocardiography be performed in all suspected cases in order to avoid both underdiagnosis and overdiagnosis. Carapetis and Currie suggest that cases are missed because some patients have only monoarthritis and not polyarthritis.[25] They would like to see monoarthritis become a major criterion. The same authors suggest that the set point of fever at 38ºC might be too high. As mentioned above, at least one author reported on atypical cases in which arthritis involved small joints rather than large joints. Finally, Rayamajhi et al suggest that arthralgia be changed from a minor to a major Jones criterion.[26]

As mentioned above, there are authorities who suggest that less stringent echocardiographic criteria for the diagnosis of rheumatic valvular disease will increase the number of cases diagnosed.[19]

Karacan et al found several asymptomatic rhythm disturbances in children with ARF. Those without carditis often had accelerated junctional rhythm. Those with carditis often had premature contractions. The group suggests a role for 24-hour electrocardiography.[27]

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Causes

Acute rheumatic fever (ARF) has been linked definitively with a preceding streptococcal infection, usually of the upper respiratory tract. Evidence is very strong that the M protein in certain streptococci subtypes is responsible for antigenicity.

Although streptococcal skin infections also are extremely common, they have not been linked with acute rheumatic fever in the United States. Note the suggestion by McDonald et al that pyoderma may be the cause in Aboriginal populations of Australia.[10]

See discussion under Pathophysiology for reference to genetic predisposition.

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Contributor Information and Disclosures
Author

Steven J Parrillo, DO, FACOEP, FACEP  Associate Professor, Emergency Medicine, Jefferson Medical College and Philadelphia College of Osteopathic Medicine; Medical Director, Department of Emergency Medicine, Einstein Elkins Park Hospital and Roxborough Memorial Hospital; Chair, Emergency Management Committee, Albert Einstein Healthcare Network; Adjunct Professor, School of Health and Science, Philadelphia University; Medical Director and Faculty, Disaster Medicine and Management Masters Program, Philadelphia University

Steven J Parrillo, DO, FACOEP, FACEP is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American Osteopathic Association, Society for Academic Emergency Medicine, and World Association for Disaster and Emergency Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Catherine V Parrillo, DO, FACOP, FAAP  Retired Clinical Assistant Professor, Department of Pediatrics, Philadelphia College of Osteopathic Medicine

Catherine V Parrillo, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Assaad J Sayah, MD  Chief, Department of Emergency Medicine, Cambridge Health Alliance

Assaad J Sayah, MD is a member of the following medical societies: National Association of EMS Physicians

Disclosure: Nothing to disclose.

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

Gino A Farina, MD, FACEP, FAAEM  Associate Professor of Clinical Emergency Medicine, Albert Einstein College of Medicine; Program Director, Department of Emergency Medicine, Long Island Jewish Medical Center

Gino A Farina, MD, FACEP, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Robert E O'Connor, MD, MPH  Professor and Chair, Department of Emergency Medicine, University of Virginia Health System

Robert E O'Connor, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American College of Physician Executives, American Heart Association, American Medical Association, Medical Society of Delaware, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Wilderness Medical Society

Disclosure: Nothing to disclose.

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