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Performance of the 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria

Eduardo Martín-Nares1, Gabriela Hernández-Molina1, Diego Federico Baenas2

  • 1From the Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases
|January 11, 2024
PubMed

Insights

The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria (AECC) show high specificity for IgG4-related disease (IgG4-RD) in Latin American patients. Adjustments to the criteria may improve sensitivity for identifying IgG4-RD.

Area of Science:

  • Rheumatology
  • Immunology
  • Clinical Diagnostics

Background:

  • The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria (AECC) are crucial for diagnosing IgG4-related disease (IgG4-RD).
  • Previous performance evaluations of the 2019 AECC primarily involved White and Asian populations.
  • A knowledge gap exists regarding the criteria's efficacy in Latin American populations.

Purpose of the Study:

  • To evaluate the performance of the 2019 AECC for IgG4-related disease (IgG4-RD) in a diverse Latin American cohort.
  • To assess the criteria's sensitivity and specificity in this underrepresented population.

Main Methods:

  • A multicenter retrospective medical record review was conducted across five Latin American countries.
  • Data from 300 patients, including those with IgG4-RD and mimicker conditions, were analyzed.
  • The 2019 AECC was applied retrospectively, with diagnosis confirmed by established comprehensive criteria.

Main Results:

  • The 2019 AECC demonstrated high specificity (100%) but moderate sensitivity (66.7%) in the Latin American cohort.
  • Removing disease-specific autoantibody items increased sensitivity to 73.3% without compromising specificity.
  • True positive cases often presented with more organ involvement and had available biopsy results.

Conclusions:

  • The 2019 AECC is highly specific for excluding non-IgG4-RD cases in Latin American populations.
  • Concomitant autoimmune diseases and non-clinically significant autoantibodies may lead to under-diagnosis by the current criteria.
  • Further refinement of the 2019 AECC may be necessary to enhance sensitivity in diverse ethnic groups.
Abstract

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