The 2019 American College of Rheumatology/European League against Rheumatism classification criteria for IgG4-related disease

Zachary S. Wallace, Ray P. Naden, Suresh Chari, Hyon K. Choi, Emanuel Della-Torre, Jean Francois Dicaire, Phillip A. Hart, Dai Inoue, Mitsuhiro Kawano, Arezou Khosroshahi, Marco Lanzillotta, Kazuichi Okazaki, Cory A. Perugino, Amita Sharma, Takako Saeki, Nicolas Schleinitz, Naoki Takahashi, Hisanori Umehara, Yoh Zen, John H. Stone

Research output: Contribution to journalReview articlepeer-review

92 Scopus citations


IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises; existing literature; derivation and validation cohorts of 1879 subjects (1086 cases, 793 mimickers); and multicriterion decision analysis to identify, weight and test potential classification criteria. Two independent validation cohorts were included. A three-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least one of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serological, radiological and pathological items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, eight weighted inclusion criteria domains, addressing clinical findings, serological results, radiological assessments and pathological interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% CI 97.2% to 99.8%) and a sensitivity of 85.5% (95% CI 81.9% to 88.5%). In the second, the specificity was 97.8% (95% CI 93.7% to 99.2%) and the sensitivity was 82.0% (95% CI 77.0% to 86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiological and basic science investigations.

Original languageEnglish (US)
Pages (from-to)77-87
Number of pages11
JournalAnnals of the rheumatic diseases
Issue number1
StatePublished - Jan 1 2020


  • Sjøgren's syndrome
  • inflammation
  • rheumatoid arthritis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology
  • General Biochemistry, Genetics and Molecular Biology


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