systemic autoimmune disease IgG4-related disease 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. DOID:0080356 GARD:0012521 IgG4-associated disease IgG4-positive multiorgan lymphoproliferative syndrome IgG4-related autoimmune disease IgG4-related sclerosing disease IgG4-related systemic disease IgG4-related systemic sclerosing disease IgG4-syndrome Immunoglobulin G4-related sclerosing disease MONDO:0017287 MedDRA:10071569 NCIT:C95992 ORDO:284264 Orphanet:284264 UMLS:C3203653 hyper-IgG4 disease multifocal fibrosclerosis multifocal idiopathic fibrosclerosis systemic IgG4-related plasmacytic syndrome systemic IgG4-related sclerosing syndrome