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


Inoue D7, Kawano M8, Khosroshahi A9, Lanzillotta M10, Okazaki K11, Perugino CA12, Sharma A13, Saeki T14, Schleinitz N15, Takahashi N16, Umehara H17, Zen Y18, Stone JH19; Members of the ACR/EULAR IgG4-RD Classification Criteria Working Group. Ann Rheum Dis. 2020 Jan;79(1):77-87. doi: 10.1136/annrheumdis-2019-216561. Epub 2019 Dec 3.

Author Information

1 Rheumatology Unit, Massachusetts General Hospital, Boston, Massachusetts, USA.

2 Maternal-Fetal Medicine, McMaster University Faculty of Health Sciences, Hamilton, Ontario, Canada.

3 Gastroenterology & Hepatology, Mayo Clinic, Rochester, Minnesota, USA.

4 Rheumatology, Harvard Medical School, Boston, Massachusetts, USA.

5 Cancer Center, Massachusetts General Hospital, Boston, Massachusetts, USA.

6 Pinnacle Inc, Montreal, Ontario, Canada.

7 Department of Radiology, Kanazawa University, Kanazawa, Japan.

8 Division of Rheumatology, Department of Internal Medicine, Kanazawa University Hospital, Kanazawa, Japan.

9 Rheumatology, Emory University School of Medicine, Atlanta, Georgia, USA.

10 Università Vita-Salute San Raffaele, School of Medicine, Unit of Internal Medicine, Ospedale San Raffaele, Milano, Lombardia, Italy.

11 Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan.

12 Rheumatology, MGH, Boston, Massachusetts, USA.

13 Radiology, Massachusetts General Hospital, Boston, Massachusetts, USA.

14 Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan.

15 Internal Medicine, Groupe hospitalier Timone, Assistance publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France.

16 Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.

17 Rheumatology and Immunology, Shiritsu Nagahama Byoin, Nagahama, Shiga, Japan.

18 Diagnostic Pathology, Kobe University, Kobe, Japan.

19 Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, Massachusetts, USA jhstone@mgh.harvard.edu.



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.