Immunoglobulin G4-related sclerosing disease
MONDO:0017287A 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
69 clinical trials for this condition and its sub-types, 30 tagged with Immunoglobulin G4-related sclerosing disease itself.
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Sub-types of Immunoglobulin G4-related sclerosing disease
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Autoimmune pancreatitis 3 trials
2 sub-types
- Autoimmune pancreatitis type 1 0 trials
- Autoimmune pancreatitis type 2 0 trials
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IgG4-related kidney disease 1 trial
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1 sub-type
- Fibrosclerosis, multifocal 0 trials
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IgG4-related aortitis 0 trials
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IgG4-related mediastinitis 0 trials
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IgG4-related mesenteritis 0 trials
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IgG4-related ophthalmic disorder 0 trials
1 sub-type
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IgG4-related pachymeningitis 0 trials
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IgG4-related sclerosing cholangitis 0 trials
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IgG4-related thyroid disease 0 trials
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Cutaneous pseudolymphoma 0 trials
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Eosinophilic angiocentric fibrosis 0 trials
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Primary cutaneous plasmacytosis 0 trials
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Gene therapy shot aims to tame autoimmune diseases in early trial
Disease control OngoingThis early-phase study tests a single injection of a gene therapy called V001-BCMA for people with autoimmune diseases like lupus that have not responded to standard treatments. The therapy targets and modifies specific immune cells to reduce disease activity. The main goal is to…
Early phase 1 • Sponsor: Tongji Hospital • Aim: Disease control
Last updated Sep 19, 2026 00:00 UTC
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Investigational drug AMG 335 offered to adults with IgG4-Related disease
Disease control ApprovedThis expanded access program allows adults with a confirmed diagnosis of IgG4-related disease to request the investigational drug AMG 335, also known as UPLIZNA. IgG4-related disease is a rare condition where the immune system causes inflammation and swelling in organs. The progr…
Sponsor: Amgen • Aim: Disease control
Last updated Sep 16, 2026 00:00 UTC
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New drug aims to shrink swollen glands in rare disease
Disease control OngoingThis study tests whether efgartigimod, a drug that lowers certain antibodies, can reduce swelling in the tear glands, saliva glands, or pancreas of people with IgG4-related disease. Five adults will receive weekly injections for 12 weeks and have regular scans and checkups. The g…
Phase 2 • Sponsor: Stanford University • Aim: Disease control
Last updated Jul 25, 2026 00:00 UTC
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New drug aims to stop Flare-Ups in rare autoimmune disease
Disease control OngoingThis study tests a drug called obexelimab to see if it can prevent flare-ups in people with IgG4-related disease, a rare condition where the immune system attacks the body's own tissues. About 194 adults with active disease will receive either the drug or a placebo. The main goal…
Phase 3 • Sponsor: Zenas BioPharma (USA), LLC • Aim: Disease control
Last updated Jun 27, 2026 09:01 UTC
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New drug aims to stop Flare-Ups in rare autoimmune disease
Disease control OngoingThis study tests a drug called inebilizumab to see if it can prevent flare-ups in people with IgG4-related disease, a rare condition that causes swelling and damage in organs. About 135 adults who have had recent flare-ups are taking part. The main goal is to see how long it take…
Phase 3 • Sponsor: Amgen • Aim: Disease control
Last updated Jun 27, 2026 08:03 UTC