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. [ NCIT:C95992 ]
Synonyms: Immunoglobulin G4-related sclerosing disease IgG4-related systemic disease IgG4-related sclerosing disease IgG4-RD
Term information
- NANDO:1200924 (https://orcid.org/0000-0003-0011-764X)
- MEDGEN:473761 (MONDO:equivalentTo)
- MedDRA:10071569 (Orphanet:284264/e)
- Orphanet:596448 (MONDO:equivalentTo)
- GARD:12521 (MONDO:GARD)
- UMLS:C3203653 (MONDO:equivalentTo)
- NANDO:1200923 (https://orcid.org/0000-0003-0011-764X)
- icd11.foundation:99883782 (https://orcid.org/0000-0002-4142-7153)
- NCIT:C95992 (MONDO:equivalentTo)
- DOID:0080356 (MONDO:equivalentTo)
- Orphanet:284264 (MONDO:equivalentTo)
ordo_disorder, gard_rare, otar, disease_grouping, rare, orphanet_rare, ordo_group_of_disorders
http://www.orpha.net/ORDO/Orphanet_596448
http://purl.obolibrary.org/obo/DOID_0080356
http://www.orpha.net/ORDO/Orphanet_284264
http://purl.obolibrary.org/obo/NCIT_C95992
http://identifiers.org/medgen/473761
http://linkedlifedata.com/resource/umls/id/C3203653
IgG4-positive multiorgan lymphoproliferative syndrome
systemic IgG4-related plasmacytic syndrome
IgG4-syndrome
systemic IgG4-related sclerosing syndrome
multifocal fibrosclerosis
IgG4-associated disease
hyper-IgG4 disease
multifocal idiopathic fibrosclerosis
IgG4-related autoimmune disease
IgG4-related systemic sclerosing disease