Immunoglobulin G4-related sclerosing disease

MONDO:0017287

A recently described mass-forming lesion that occurs in the pancreas, submandibular glands, lacrimal glands, lymph nodes, and hepatobiliary tract. It is characterized by the presence of marked tissue sclerosis and infiltration by numerous plasma cells. The plasma cells show immunohistochemical staining for IgG4 and the serum IgG4 levels are often increased.

Also known as: IgG4-RD, IgG4-related disease, IgG4-related sclerosing disease, IgG4-related systemic disease, immunoglobulin G4-related sclerosing disease, IgG4-associated disease, IgG4-positive multiorgan lymphoproliferative syndrome, IgG4-related autoimmune disease

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