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 2 The updated 2020 Japanese Comprehensive Clinical Diagnostic (CCD) criteria for IgG4-related disease [20].
| Clinical, radiological, serological, and histological criteria for IgG4-related disease | |
|---|---|
| * Indicates that this is not a pathognomonic finding and may be present in any chronic inflammatory disease. | |