immunoglobulin G4-related sclerosing disease
Findings
No curated finding names immunoglobulin G4-related sclerosing disease yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
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.
Definition from the Mondo Disease Ontology (MONDO:0017287), read 2026-09-29. CC BY 4.0.
Features
18 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal pancreatic duct morphologyHPOHP:0030992
- Frequent (30% to 79% of cases)
- Abnormality of the orbital regionHPOHP:0000315
- Frequent (30% to 79% of cases)
- Antinuclear antibody positivityHPOHP:0003493
- Frequent (30% to 79% of cases)
- Decreased circulating complement C3 concentrationHPOHP:0005421
- Frequent (30% to 79% of cases)
- Decreased circulating complement C4 concentrationHPOHP:0045042
- Frequent (30% to 79% of cases)
- Hashimoto thyroiditisHPOHP:0000872
- Frequent (30% to 79% of cases)
Show the remaining 6
- Elevated circulating C-reactive protein concentrationHPOHP:0011227
- Occasional (5% to 29% of cases)
- Elevated erythrocyte sedimentation rateHPOHP:0003565
- Occasional (5% to 29% of cases)
- Increased circulating IgE concentrationHPOHP:0003212
- Occasional (5% to 29% of cases)
- Increased circulating IgG concentrationHPOHP:0003237
- Occasional (5% to 29% of cases)
- Rheumatoid factor positiveHPOHP:0002923
- Occasional (5% to 29% of cases)
- GranulomaHPOHP:0032252
Where it sits
- A kind of
- Narrower terms (14)
- autoimmune pancreatitis
- cutaneous pseudolymphoma
- eosinophilic angiocentric fibrosis
- IgG4-related aortitis
- IgG4-related kidney disease
- IgG4-related mediastinitis
- IgG4-related mesenteritis
- IgG4-related ophthalmic disorder
- IgG4-related pachymeningitis
- IgG4-related retroperitoneal fibrosis
- IgG4-related sclerosing cholangitis
- IgG4-related submandibular gland disease
- IgG4-related thyroid disease
- primary cutaneous plasmacytosis
Other names
4 names
Resolves to: immunoglobulin G4-related sclerosing disease
- Also called
- IgG4-RDIgG4-related diseaseIgG4-related sclerosing diseaseIgG4-related systemic disease