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

© 2023. BioMed Central Ltd., part of Springer Nature..

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2023

Erschienen:

2023

Enthalten in:

Zur Gesamtaufnahme - volume:25

Enthalten in:

Arthritis research & therapy - 25(2023), 1 vom: 05. Sept., Seite 163

Sprache:

Englisch

Beteiligte Personen:

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

Links:

Volltext

Themen:

Criteria
IgG4-RD
Journal Article
Phenotypes
Research Support, Non-U.S. Gov't

Anmerkungen:

Date Completed 07.09.2023

Date Revised 18.11.2023

published: Electronic

Citation Status MEDLINE

doi:

10.1186/s13075-023-03155-y

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM361680651