The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease

for the American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group

Research output: Contribution to journalArticle

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Abstract

Objective: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. Methods: 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 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results: A 3-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2–99.8%) and a sensitivity of 85.5% (95% CI 81.9–88.5%). In the second, the specificity was 97.8% (95% CI 93.7–99.2%) and the sensitivity was 82.0% (95% CI 77.0–86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion: 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, epidemiologic, and basic science investigations.

Original languageEnglish (US)
JournalArthritis and Rheumatology
DOIs
StateAccepted/In press - Jan 1 2019

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Immunoglobulin G
Confidence Intervals
Decision Support Techniques
Rheumatology
Rheumatic Diseases
Radiology
Research Personnel
Exercise
Pathology
Physicians
Weights and Measures

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology

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for the American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group (Accepted/In press). The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis and Rheumatology. https://doi.org/10.1002/art.41120

The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. / for the American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group.

In: Arthritis and Rheumatology, 01.01.2019.

Research output: Contribution to journalArticle

for the American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group 2019, 'The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease', Arthritis and Rheumatology. https://doi.org/10.1002/art.41120
for the American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis and Rheumatology. 2019 Jan 1. https://doi.org/10.1002/art.41120
for the American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group. / The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. In: Arthritis and Rheumatology. 2019.
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abstract = "Objective: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. Methods: 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 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results: A 3-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2{\%} (95{\%} confidence interval [95{\%} CI] 97.2–99.8{\%}) and a sensitivity of 85.5{\%} (95{\%} CI 81.9–88.5{\%}). In the second, the specificity was 97.8{\%} (95{\%} CI 93.7–99.2{\%}) and the sensitivity was 82.0{\%} (95{\%} CI 77.0–86.1{\%}). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion: 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, epidemiologic, and basic science investigations.",
author = "{for the American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group} and Wallace, {Zachary S.} and Naden, {Ray P.} and Suresh Chari and Hyon Choi and Emanuel Della-Torre and Dicaire, {Jean Francois} and Hart, {Phil A.} and Dai Inoue and Mitsuhiro Kawano and Arezou Khosroshahi and Kensuke Kubota and Marco Lanzillotta and Kazuichi Okazaki and Perugino, {Cory A.} and Amita Sharma and Takako Saeki and Hiroshi Sekiguchi and Nicolas Schleinitz and Stone, {James R.} and Naoki Takahashi and Hisanori Umehara and George Webster and Yoh Zen and Stone, {John H.}",
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AU - for the American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group

AU - Wallace, Zachary S.

AU - Naden, Ray P.

AU - Chari, Suresh

AU - Choi, Hyon

AU - Della-Torre, Emanuel

AU - Dicaire, Jean Francois

AU - Hart, Phil A.

AU - Inoue, Dai

AU - Kawano, Mitsuhiro

AU - Khosroshahi, Arezou

AU - Kubota, Kensuke

AU - Lanzillotta, Marco

AU - Okazaki, Kazuichi

AU - Perugino, Cory A.

AU - Sharma, Amita

AU - Saeki, Takako

AU - Sekiguchi, Hiroshi

AU - Schleinitz, Nicolas

AU - Stone, James R.

AU - Takahashi, Naoki

AU - Umehara, Hisanori

AU - Webster, George

AU - Zen, Yoh

AU - Stone, John H.

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N2 - Objective: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. Methods: 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 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results: A 3-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2–99.8%) and a sensitivity of 85.5% (95% CI 81.9–88.5%). In the second, the specificity was 97.8% (95% CI 93.7–99.2%) and the sensitivity was 82.0% (95% CI 77.0–86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion: 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, epidemiologic, and basic science investigations.

AB - Objective: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. Methods: 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 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results: A 3-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2–99.8%) and a sensitivity of 85.5% (95% CI 81.9–88.5%). In the second, the specificity was 97.8% (95% CI 93.7–99.2%) and the sensitivity was 82.0% (95% CI 77.0–86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion: 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, epidemiologic, and basic science investigations.

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