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 4 Current status of biomarkers used in routine laboratory testing for active or recurrent IgG4-related disease [21].
| Biomarker | Test levels |
|---|---|
| • IgG4 | ↑ |
| • IgE | ↑ |
| • Complement C3 and C4 | ↓ |
| • Eosinophil count | ↑ |
| • Erythrocyte sedimentation rate (ESR) | ↑ |
| • Plasma cell count | ↑ |
| • Memory B cell count | ↑ |
| • Lipase | ↑ |
| • Alanine transaminase | ↑ |
| • Aspartate transaminase | ↑ |
| • Alkaline phosphatase | ↑ |
| • Gamma-glutamyl transferase | ↑ |
| • Bilirubin | ↑ |
| • Creatinine | ↑ |
| • Total urinary protein to creatinine ratio | ↑ |
| * All are non-specific markers of inflammation that may be associated with chronic or acute on chronic inflammation (primary or secondary). | |