FRI0503 VALIDATION OF THE 2019 ACR/EULAR CLASSIFICATION CRITERIA FOR IGG4-RELATED DISEASE IN A JAPANESE KIDNEY DISEASE COHORT: A MULTI-CENTER RETROSPECTIVE STUDY BY THE IGG4-RELATED KIDNEY DISEASE (IGG4-RKD) WORKING GROUP OF THE JAPANESE SOCIETY OF NEPHROLOGY. (2nd June 2020)

• Record Type: Journal Article
• Authors: Saeki, T.; Kawano, M.; Nagasawa, T.; Ubara, Y.; Taniguchi, Y.; Yanagita, M.; Nishi, S.; Nagata, M.; Yamaguchi, Y.; Saito, T.; Nakashima, H.
• Is Part Of: Annals of the rheumatic diseases. Volume 79(2020)Supplement 1
• Page Start: 849
• Page End: 850
• Publication Date: 2020-06-02
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2020-eular.1097
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: The 2019 ACR/EULAR classification criteria for IgG4-RD have recently been published 1) . In the criteria, patients with an inclusion criteria score of >20 without exclusion criteria are classified as having IgG4-RD. Objectives: To validate the 2019 ACR/EULAR classification criteria for IgG4-RD in a Japanese kidney disease cohort. Methods: The study involved Japanese patients diagnosed as having kidney disease between April 2012 and May 2019, for whom sufficient clinical information and data on serum IgG4 values and/or immunohistological staining for IgG4 in renal biopsy samples were known. These patients were classified as having IgG4-RKD or non-IgG4-RKD (mimickers) based on the 2019 ACR/EULAR classification criteria for IgG4-RD, and the results were evaluated by expert opinion. Results: Among 105 included patients, the expert panel diagnosed 55 as having true IgG4-RKD and 50 as mimickers. The final diagnoses among the mimickers were vasculitis (n=11), idiopathic tubulointerstitial nephritis (TIN) (n=5), drug-induced TIN (n=5), Sjögren's syndrome (n=4) and others. Among the 55 true IgG4-RKD patients, 4 had exclusion criteria, and 50 of the remaining 51 had an inclusion criteria score of ≥20 points (sensitivity 90.9%). On the other hand, 49 of the 50 mimickers were classified as having non-IgG4-RKD (specificity 98.0%) (Table 1 ). Conclusion: The 2019 ACR/EULAR classification criteria for IgG4-RD showed good agreement with expert classification in this Japanese kidney disease cohort. References: [1] Wallace ZS, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 79:77-87, 2020 Disclosure of Interests: None declared