Pancreas and head-and-neck regions dominate in IgG4-related disease cases
A comprehensive Japanese registry study of 808 confirmed IgG4-related disease (IgG4-RD) cases has provided valuable insights into the clinical profile, commonly affected organs, diagnostic criteria, and pathological features of the disease. The pancreas and head-and-neck regions are reaffirmed as major areas of involvement, with findings aimed at improving future diagnosis and management of IgG4-RD.
Key findings include:
- Demographics: The mean age of patients was 67.9 years, with a male predominance (68.8%).
- Affected Organs: The pancreas was the most commonly involved organ (49.8%), followed by submandibular glands (46.2%) and lacrimal glands (30.6%).
- Co-occurrence: Submandibular adenitis and autoimmune pancreatitis were frequently observed in the same patients, though no direct association was found between these organs.
- Diagnostic Criteria: Comprehensive diagnostic criteria were used in 63.6% of cases.
- Pathology: Storiform fibrosis and phlebitis obliterans exhibited varying frequencies depending on the organ involved.
This study underscores the importance of the pancreas and head-and-neck region in IgG4-RD and offers valuable data on clinical, imaging, and pathological characteristics that will inform future diagnostic and treatment approaches.
Reference
Yamamoto M, Kanda M, Mizushima I, et al; Clinical profile of IgG4-related disease in Japan based on the rare disease data registry. Immunol Med. 2024;doi: 10.1080/25785826.2024.2430812. Epub ahead of print. PMID: 39607031.
