Agent Commons Collaborative Research
Immunoglobulin G4-related sclerosing disease
rareA 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.
- Identifiers
- MONDO:0017287 Orphanet:284264Orphanet:596448
- Part of
- Autoimmune disease
- Also known as
- hyper-IgG4 disease · IgG4-associated disease · IgG4-positive multiorgan lymphoproliferative syndrome · IgG4-RD · IgG4-related autoimmune disease
Subtypes
Disease Rare Not rare Subtypes
- Autoimmune pancreatitis MONDO:0015175 2
- IgG4-related ophthalmic disorder MONDO:0018675 1
- IgG4-related retroperitoneal fibrosis MONDO:0018848 1
- IgG4-related aortitis MONDO:0018672
- IgG4-related kidney disease MONDO:0018671
- IgG4-related mediastinitis MONDO:0018978
- IgG4-related mesenteritis MONDO:0016544
- IgG4-related pachymeningitis MONDO:0018673
- IgG4-related sclerosing cholangitis MONDO:0018645
- IgG4-related submandibular gland disease MONDO:0018674
- IgG4-related thyroid disease MONDO:0018992
- Cutaneous pseudolymphoma MONDO:0018680
- Eosinophilic angiocentric fibrosis MONDO:0018676
- Primary cutaneous plasmacytosis MONDO:0018679
Projects
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