Differential sensitivity of the 2020 revised comprehensive diagnostic criteria and the 2019 ACR/EULAR classification criteria across IgG4-related disease phenotypes: results from a Norwegian cohort

Jens Vikse; Øyvind Midtvedt; Bjørg-Tilde Svanes Fevang; Torhild Garen; Øyvind Palm; Marianne Wallenius; Gunnstein Bakland; Katrine Brække Norheim; Øyvind Molberg; Anna-Maria Hoffmann-Vold

doi:10.1186/s13075-023-03155-y

Differential sensitivity of the 2020 revised comprehensive diagnostic criteria and the 2019 ACR/EULAR classification criteria across IgG4-related disease phenotypes: results from a Norwegian cohort

Arthritis Res Ther. 2023 Sep 5;25(1):163. doi: 10.1186/s13075-023-03155-y.

Authors

Jens Vikse^{1

2}, Øyvind Midtvedt³, Bjørg-Tilde Svanes Fevang^{4

5}, Torhild Garen³, Øyvind Palm³, Marianne Wallenius^{6

7}, Gunnstein Bakland⁸, Katrine Brække Norheim^{4

9}, Øyvind Molberg^{3

10}, Anna-Maria Hoffmann-Vold^{3

11}

Affiliations

¹ Department of Clinical Science, University of Bergen, Jonas Lies Veg 87, 5021, Bergen, Norway. Jens.vikse@uib.no.
² Department of Rheumatology, Stavanger University Hospital, Stavanger, Norway. Jens.vikse@uib.no.
³ Department of Rheumatology, Oslo University Hospital, Oslo, Norway.
⁴ Department of Clinical Science, University of Bergen, Jonas Lies Veg 87, 5021, Bergen, Norway.
⁵ Department of Rheumatology, Haukeland University Hospital, Bergen, Norway.
⁶ Department of Rheumatology, St. Olav University Hospital, Trondheim, Norway.
⁷ Department of Neuromedicine and Movement Science, Norwegian University of Science and Technology, Trondheim, Norway.
⁸ Department of Rheumatology, University Hospital of North Norway, Tromsø, Norway.
⁹ Department of Rheumatology, Stavanger University Hospital, Stavanger, Norway.
¹⁰ Department of Clinical Medicine, University of Oslo, Oslo, Norway.
¹¹ Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.

Abstract

Background: We investigated sensitivity of the 2020 Revised Comprehensive Diagnostic Criteria (RCD) and the 2019 ACR/EULAR classification criteria across the four identified IgG4-related disease (IgG4-RD) phenotypes: "Pancreato-Hepato-Biliary", "Retroperitoneum and Aorta", "Head and Neck-limited" and "Mikulicz' and Systemic" in a well-characterized patient cohort.

Methods: We included adult patients diagnosed with IgG4-RD after comprehensive clinical assessment at Oslo University Hospital in Norway. We assigned patients to IgG4-RD phenotypes based on pattern of organ involvement and assessed fulfillment of RCD and 2019 ACR/EULAR classification criteria. Differences between phenotype groups were analyzed using one-way ANOVA for continuous variables, and contingency tables for categorical variables.

Results: The study cohort included 79 IgG4-RD patients assigned to the "Pancreato-Hepato-Biliary" (22.8%), Retroperitoneum and Aorta" (22.8%) "Head and Neck-limited" (29.1%), and "Mikulicz' and Systemic" (25.3%) phenotype groups, respectively. While 72/79 (91.1%) patients in total fulfilled the RCD, proportion differed across phenotype groups and was lowest in the "Retroperitoneum and Aorta" group (66.7%, p < 0.001). Among the 57 (72.2%) patients meeting the 2019 ACR/EULAR classification criteria, proportion was again lowest in the "Retroperitoneum and Aorta" group (27.8%, p < 0.001).

Conclusion: The results from this study indicate that IgG4-RD patients having the "Retroperitoneum and Aorta" phenotype less often fulfill diagnostic criteria and classification criteria than patients with other IgG4-RD phenotypes. Accordingly, this phenotype is at risk of being systematically selected against in observational studies and randomized clinical trials, with potential implications for patients, caregivers and future definitions of IgG4-RD.

Keywords: Criteria; IgG4-RD; Phenotypes.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Humans
Immunoglobulin G4-Related Disease*
Norway
Phenotype