Diagnosing Acute Rheumatic Fever

Key points

  • There's no definitive diagnostic test for acute rheumatic fever.
  • A clinical diagnosis of acute rheumatic fever should be made using the 2015 revised Jones Criteria.
  • The Jones Criteria can be used to diagnose initial as well as recurrent illnesses.
A doctor looks over results from a child’s electrocardiogram

Differential diagnosis

The differential diagnosis of acute rheumatic fever is broad due to the various symptoms of the disease. The differential diagnosis may include specific autoimmune diseases, inflammatory diseases, cancers, and other conditions1.

Autoimmune diseases

  • Juvenile idiopathic arthritis

  • Rheumatoid arthritis
  • Systemic lupus erythematosus

    • Cancers

  • Leukemia
  • Hodgkin's disease

    • Inflammatory diseases

  • Gout
  • Henoch-Schonlein purpura
  • Infective endocarditis
  • Sarcoidosis
  • Septic arthritis
  • Viral myocarditis

    • Other conditions

  • Lyme disease
  • Serum sickness

    • Jones criteria

    There's no definitive diagnostic test for acute rheumatic fever. Use the Jones criteria to make a clinical diagnosis of acute rheumatic fever.

    Defining risk populations per revised Jones criteria

    Low-risk population

    • An acute rheumatic fever incidence of <2 per 100,000 school-age children
    • All age rheumatic heart disease prevalence of ≤1 per 1000 population per year

    Moderate- and high-risk populations

    Those not included in the low-risk population are defined as moderate or high risk depending upon their reference population.

    Classifying manifestations by population

    Low-risk populations

    Major manifestations

    • Carditis (clinical or subclinical)
    • Arthritis (polyarthritis only)
    • Chorea
    • Erythema marginatum
    • Subcutaneous nodules

    Minor manifestations

    • Polyarthralgia
    • Fever (≥38.5oC)
    • Elevated acute phase reactants (ESR ≥60 mm in the first hour or CRP ≥3.0 mg/dl)
    • Prolonged PR interval on electrocardiography, after accounting for age variability (unless carditis is a major criterion)

    Moderate- and high-risk populations

    Major manifestations

    • Carditis (clinical or subclinical)
    • Arthritis (monoarthritis or polyarthritis, as well as polyarthralgia if other causes have been excluded)
    • Chorea
    • Erythema marginatum
    • Subcutaneous nodules

    Minor manifestations

    • Monoarthralgia
    • Fever (≥38.5oC)
    • Elevated acute phase reactants (ESR ≥30 mm/hour or CRP >3.0 mg/dl)
    • Prolonged PR interval on electrocardiography, after accounting for age variability (unless carditis is a major criterion)

    Abbreviations

    ESR = erythrocyte sedimentation rate
    CRP = C-reactive protein
    mm = millimeters
    mg/dl = milligrams per deciliter

    Initial acute rheumatic fever illness

    Criteria

    The presence of the following indicates a high probability of an initial acute rheumatic fever illness in any risk population:

    • 2 major manifestations
    • 1 major and 2 minor manifestations

    More than 1 joint manifestation: Classify them as either one major or one minor criteria, not both. For example, if there's evidence of arthritis (a major criteria), then arthralgia doesn't count as a minor criteria.

    More than 1 cardiac manifestation: Classify them as either one major or one minor criteria, not both. For example, if there's evidence of carditis (a major criteria), then a prolonged PR interval doesn't count as a minor criteria.

    Preceding infection

    In most cases, there should also be evidence of preceding group A streptococcal infection. Evidence to support an antecedent group A strep infection include:

    • Positive throat culture or rapid streptococcal antigen test
    • Elevated or rising streptococcal antibody titer

    Presumptive diagnosis

    In some instances, a presumptive diagnosis of acute rheumatic fever can be made without fulfilling the Jones Criteria.

    Use clinical judgement regarding diagnosis and antibiotic prophylaxis in areas of high acute rheumatic fever incidence when lacking clinical evidence2.

    Acute rheumatic fever can be considered in cases of chorea and indolent, chronic carditis. This can be the case despite the lack of group A streptococcal laboratory confirmation or fullfillment of Jones criteria2.

    Recurrent disease

    Additional episodes can occur with re-exposure

    Individuals with a history of rheumatic heart disease or prior episode of acute rheumatic fever are at increased risk for recurrences of acute rheumatic fever.

    Criteria

    A presumptive diagnosis of a recurrence can be made with any of the following:

    • 2 major manifestations
    • 1 major and 2 minor manifestations
    • 3 minor manifestations

    Relying on 3 minor manifestations: Make the recurrent acute rheumatic fever diagnosis only after more likely causes have been excluded.

    Preceding infection

    There should be a preceding group A streptococcal infection documented.

    Recommended supplemental tests

    Routine echocardiography/Doppler is now recommended for all confirmed or suspected acute rheumatic fever cases2.

    This recommendation applies regardless of the presence or absence of murmur on physical exam.

    Resources

    Diagnosis guidelines


    American Heart Association


    World Heart Federation

    Print
    Content Source:
    National Center for Immunization and Respiratory Diseases; Division of Bacterial Diseases
    1. Shulman ST, Bisno AL. Nonsupprative poststreptococcal sequelae: Rheumatic fever and glomerulonephritis. In Bennett J, Dolin R, Blaser M, editors. 8th Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. Philadelphia (PA): Elsevier. 2015;2:2300–9.
    2. Gewitz MH, Baltimore RS, Tani LY, et al. . Circulation. 2015;131:1806.