IgG4-Related Sclerosing Disease

Abstract

We report to cases of systemic IgG4-related sclerosing disease involving the dissemination of CD4- or CD8-positive T lymphocytes and IgG4-positive plasma cells in numerous internal organs. The pancreas, bile duct, salivary glands, thyroid gland, and retroperitoneum are often permeated by IgG4-positive plasma cells, suggesting that sclerosing salivary glanditis as Mikulicz’s disease and Küttner’s tumor, autoimmune pancreatitis, sclerosing cholangitis, and retroperitoneal fibrosis are IgG4-related sclerosing phenotypes. The paucity of cases has prevented development of a conclusive treatment regimen, although oral steroid is have proven effective.