18 June 2025 : Review article
IgG4-Related Disease: A Review of Persistent Challenges in the Pathogenesis, Diagnosis, and Approaches to Treatment
Dinah V. Parums CDEF 1DOI: 10.12659/MSM.950212
Med Sci Monit 2025; 31:e950212
Introduction
Epidemiology and Clinical Presentation of IgG4-Related Disease
Criteria for the Diagnosis of IgG4-Related Disease
Pathogenesis: Role of B Cells and T Cells
Diagnosis: Laboratory and Imaging Findings
Diagnosis: Histopathology
Differential Diagnoses
Disease Biomarkers
Treatment of Acute Disease and Management of Remissions
Periaortic Chronic Inflammation, Retroperitoneal Fibrosis, and Chronic Periaortitis
Future Directions
Conclusions
References
Table 3 Entry and exclusion criteria: The 2019 American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) criteria for the diagnosis of IgG4-related disease [21].
| Steps 1 to 4 | Criteria |
|---|---|
| (Yes or No) | |
| (Yes or No) | |
| Modified from: Wallace ZS, Naden RP, Chari S, et al; American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis Rheumatol. 2020;72(1):7–19. Available from: | |