eMedicine Specialties > Rheumatology > Miscellaneous Inflammatory Arthritis

Acute Rheumatic Fever: Differential Diagnoses & Workup

Author: Robert J Meador, MD, Rheumatology Fellow, Department of Rheumatology, Baylor Garland Family Practice Clinic
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
Contributor Information and Disclosures

Updated: Jul 31, 2009

Differential Diagnoses

Kawasaki Disease
Sepsis, Bacterial
Septic Arthritis
Systemic Lupus Erythematosus

Other Problems to Be Considered

Bacterial endocarditis
Still disease - Systemic-onset juvenile rheumatoid arthritis or adult Still disease
Vasculitis

Poststreptococcal reactive arthritis

Barash et al performed a retrospective study to compare clinical and laboratory features of acute rheumatic fever (ARF) versus poststreptococcal reactive arthritis to determine if the two diseases are separate clinical manifestations of the same disease or are in fact different diseases altogether. Based on a review of 68 patients with ARF and 159 patients with poststreptococcal reactive arthritis, the authors concluded that at least 4 factors differed significantly enough to show that the two diseases are distinct—ESR, C-reactive protein levels, duration of joint symptoms after initiation of anti-inflammatory treatment, and relapse of joint symptoms after treatment cessation. Using the differentiating factors, they were able to determine the correct diagnosis in more than 80% of cases.5

Workup

Laboratory Studies

  • Acute rheumatic fever (ARF) is diagnosed based on clinical manifestations supported by laboratory tests.
  • Group A streptococcal antigen detection tests are specific but not very sensitive.
  • In contrast, antistreptococcal antibodies usually reach a peak titer (in Todd units) at the time of onset of rheumatic fever and are more useful.
    • Specific antibodies to streptococcal antigens also indicate true infection rather than mere carriage of the organism. However, note that children without ARF may have an isolated positive antistreptolysin O (ASO) titer. This may also be found in patients with certain related diseases such as rheumatoid arthritis and Takayasu arteritis. Therefore, rising ASO titers should be combined with a careful clinical evaluation and the discovery of other antistreptococcal antibodies to support the diagnosis of ARF.
    • Antistreptococcal antibodies include ASO, antideoxyribonuclease B (anti-DNAse B), antistreptokinase, antihyaluronidase, and anti-DNAase (anti-DNPase).
    • These antibodies target extracellular products produced by streptococci.
    • Although age, geographic location, and season affect the titers, an elevated titer of at least one of these antibodies indicates streptococcal infection in 95% of patients.
    • ASO is found in 80-85% of patients with ARF.
  • The sensitivity of throat culture as evidence of recent streptococcal infection is 25-40%.
    • For comparison, the sensitivity of ASO titer (adults with >240 Todd U and children with >320 Todd U) is 80%.
    • The sensitivity of an elevated ASO titer in addition to anti-DNAse B or antihyaluronidase is 90%.
  • Acute-phase reactants such as C-reactive protein and ESR are usually elevated and helpful in monitoring disease activity.
  • Other laboratory tests may be helpful but not for definitive diagnosis. Synovial fluid analysis reveals a sterile inflammatory reaction, usually with fewer than 20,000 cells/μL (mainly polymorphonuclear) without crystals.

Imaging Studies

  • Echocardiography is more sensitive than standard auscultation for helping detect regurgitant lesions, but the prognostic significance of these subauscultory findings is unclear.
  • Standard auscultation is favored for detecting carditis and can reveal mitral regurgitation in as many as 80% of patients.
  • Chest radiograph may reveal cardiomegaly.

    Chest radiograph showing cardiomegaly due to card...

    Chest radiograph showing cardiomegaly due to carditis of acute rheumatic fever.

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    Chest radiograph showing cardiomegaly due to card...

    Chest radiograph showing cardiomegaly due to carditis of acute rheumatic fever.

Other Tests

  • ECG is helpful for diagnosing carditis and may reveal a prolonged PR interval, but this finding is not necessarily associated with later cardiac sequelae.
  • Conventional throat swab cultures may demonstrate streptococcal organisms.

Procedures

  • Synovial tissue biopsy is rarely performed.
  • Endomyocardial biopsies have not contributed significantly to diagnosis thus far.

Histologic Findings

Synovial biopsy reveals mild inflammatory changes. The synovial membrane may be thickened, erythematous, and covered by a fibrinous exudate. Focal fibrinoid lesions in the heart and histiocytic granulomas called Aschoff nodules may be late findings. Pancarditis develops with involvement of all layers of the heart. Subcutaneous nodule histopathology reveals edema, fibrinoid necrosis, and mononuclear cell infiltrate.

More on Acute Rheumatic Fever

Overview: Acute Rheumatic Fever
Differential Diagnoses & Workup: Acute Rheumatic Fever
Treatment & Medication: Acute Rheumatic Fever
Follow-up: Acute Rheumatic Fever
Multimedia: Acute Rheumatic Fever
References
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References

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Further Reading

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Keywords

acute rheumatic fever, ARF, group A streptococcal pharyngitis, streptococcal pharyngitis, Sydenham chorea, painful migratory arthritis, rheumatic heart disease, chorea, erythema marginatum, subcutaneous nodules, antistreptococcal antibodies, antistreptolysin O, ASO, Aschoff nodules, carditis, Jones criteria, valvular murmurs, streptococcal infection, migratory polyarthritis

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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; Jazz Pharma Grant/research funds Independent contractor; Jazz Pharma Consulting fee Board membership; Pfizer Pharma Grant/research funds Independent contractor; Pfizer Pharma Consulting fee Consulting; Pfizer Pharma Consulting fee Board membership; Lily Pharma  Independent contractor; Lily Pharma Consulting fee Speaking and teaching; Pfizer Pharma Consulting fee Speaking and teaching; Forest Laboratories Pharma Consulting fee Speaking and teaching

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, 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 of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, 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, Professor of Medicine, Temple University 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: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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