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Abstract Number: 1888

Usefulness of 2019 ACR/EULAR Classification Criteria (AECC) for IgG4-Related Disease Differs Between Clinical Phenotypes of IgG4-RD

Gözde Kübra Yardımcı1, Bayram Farisogulları2, Gizem Ayan1, Levent Kilic1, Sule Apraş Bilgen1 and Omer Karadag3, 1Hacettepe University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey, 2Division of Rheumatology, Department of Internal Medicine Faculty of Medicine, Hacettepe University, Ankara, Turkey, 3Hacettepe University, Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara, Turkey

Meeting: ACR Convergence 2021

Keywords: American College of Rheumatology Criteria, classification criteria, IgG4 Related Disease

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Session Information

Date: Tuesday, November 9, 2021

Session Title: Vasculitis – Non-ANCA-Associated & Related Disorders Poster II (1862–1888)

Session Type: Poster Session D

Session Time: 8:30AM-10:30AM

Background/Purpose: ACR/EULAR Classification criteria for IgG4-related disease (IgG4-RD) newly published [1]. On the other hand, four distinctive phenotypes of IgG4-RD have been described [2]. We aimed to identify clinical phenotypes of IgG4-RD and the evaluate usefulness of 2019 ACR/EULAR classification criteria (AECC) for clinical phenotypes in our cohort.

Methods: In the prospective database of the Hacettepe University Vasculitis Research Centre (HUVAC) 92 IgG4-RD patients meeting the 2011 comprehensive diagnostic criteria for IgG4-RD were registered by the end of the May 2021. We reviewed the medical records and determined the following clinical factors: Age, gender, serum levels of IgG4, presence of ANCA or other specific autoantibodies, histopathologies with immunostaining (if available) and distributions of organ involvement. An AECC score was calculated for all cohort.

Results: We included 92 patients (M/F:47/45) with IgG4-RD. 61% of the patients had multiorgan involvement, median (IQR) organ involvement was 2 (1-3) and median (IQR) IgG4 level was 179 mg/dL (89-368). Majority of the patients had retroperitoneal fibrosis/periaortitis (Table). Retroperitoneal and aorta (RPF-Aortitis) accounted for 40% of the phenotypes, followed by head and neck (24%), Mikulicz and systemic (16%), and pancreato-hepato-biliary (PHB) (11%). Nine per cent had an undefined phenotype. Head and neck group had female predominance whereas RPF/aortitis group had male predominance. Mikulicz and systemic group had the highest IgG4 levels.

All of the patients fulfilled the entry criteria for AECC for IgG4-RD. Of the 42 false-negative cases, 7 met one exclusion criteria whereas 35 did not achieve sufficient inclusion criteria scores. 49 (53%) patients got a score ≥20 points, with a mean score of 29.3 points (SD.7.3). MS, PHB and RPF/Aortitis phenotypes met 2019 AECC at a higher rate, although the differences were not statistically significant.

Conclusion: Phenotype results of our cohort are concordant with Asian and American cohorts. RPF-Aortitis phenotype was more prevalent, while PHB and MS were less frequent. The 2019 AECC was met over half of the patients.

Table. Clinical Phenotypes of IgG4-related disease


Disclosures: G. Yardımcı, None; B. Farisogulları, None; G. Ayan, None; L. Kilic, None; S. Apraş Bilgen, None; O. Karadag, Omer Karadag has received research grants from Roche, Pfzer as study investigator and received consulting fees from Celltrion., 6.

To cite this abstract in AMA style:

Yardımcı G, Farisogulları B, Ayan G, Kilic L, Apraş Bilgen S, Karadag O. Usefulness of 2019 ACR/EULAR Classification Criteria (AECC) for IgG4-Related Disease Differs Between Clinical Phenotypes of IgG4-RD [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/usefulness-of-2019-acr-eular-classification-criteria-aecc-for-igg4-related-disease-differs-between-clinical-phenotypes-of-igg4-rd/. Accessed February 3, 2023.
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