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

2015 revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography
American Heart Association

Criteria for echocardiographic diagnosis of rheumatic heart disease—An evidence-based guideline
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. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: A scientific statement from the American Heart Association. Circulation. 2015;131:1806.