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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New pill aims to tame rare inflammatory disease
Disease control Recruiting nowThis phase 3 study tests whether the oral drug rilzabrutinib can prevent disease flares in adults with active IgG4-related disease, a rare condition causing swelling and organ damage. About 124 participants will receive either rilzabrutinib or a placebo for 52 weeks, alongside st…
Phase 3 • Sponsor: Sanofi • Aim: Disease control
Last updated Jul 30, 2026 00:00 UTC
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Engineered immune cells take aim at autoimmune kidney diseases
Disease control Recruiting nowThis early-stage trial tests a new therapy called FKC289, which uses a patient's own immune cells engineered to target two proteins (BCMA and CD19) on cells that drive autoimmune attacks. The goal is to see if it is safe and can help control kidney diseases like lupus nephritis a…
Phase 1 • Sponsor: Nanjing University School of Medicine • Aim: Disease control
Last updated Jul 08, 2026 00:00 UTC
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Old drug, new hope: could filgotinib tame rare immune diseases?
Disease control Recruiting nowThis phase 2 trial is testing whether filgotinib, a drug already used for arthritis, can help people with three rare immune diseases: Behçet's disease, myositis, and IgG4-related disease. The goal is to see if it can control disease activity and reduce the need for long-term ster…
Phase 2 • Sponsor: UMC Utrecht • Aim: Disease control
Last updated Jun 27, 2026 12:31 UTC
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New hope for IgG4 patients: which drug combo works best?
Disease control Recruiting nowThis study tests two treatment plans for people with IgG4-related disease affecting superficial organs like glands or skin. One group gets prednisone plus iguratimod, the other gets prednisone plus leflunomide. Over 12 months, researchers will track how many patients have a relap…
Sponsor: Peking Union Medical College Hospital • Aim: Disease control
Last updated Jun 27, 2026 12:04 UTC
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New cell therapy trial aims to tame rare autoimmune disease
Disease control Recruiting nowThis early-stage trial tests a new treatment called ACE1831 for people with IgG4-related disease, a rare condition where the immune system attacks the body, causing swelling and organ damage. ACE1831 is made from healthy donor cells and given as an infusion. The study will check …
Phase 1/2 • Sponsor: Acepodia Biotech, Inc. • Aim: Disease control
Last updated Jun 27, 2026 09:04 UTC
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New drug combo aims to tame rare inflammatory disease
Disease control Recruiting nowThis study tests whether adding lenalidomide to standard steroid treatment can better control active IgG4-related disease (IgG4-RD), a rare condition that causes swelling and damage in organs. About 198 adults will receive either lenalidomide plus prednisone or prednisone alone f…
Phase 2/3 • Sponsor: Peking Union Medical College Hospital • Aim: Disease control
Last updated Jun 27, 2026 08:07 UTC
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Experimental CAR-T therapy targets stubborn autoimmune disease
Disease control Recruiting nowThis Phase 2 trial tests a personalized cell therapy called anti-BCMA-CD19 CAR-T cells in 9 adults with IgG4-related disease that hasn't responded to standard treatments. Participants receive an infusion of their own modified immune cells after a short course of chemotherapy. The…
Phase 2 • Sponsor: Chinese PLA General Hospital • Aim: Disease control
Last updated Jun 26, 2026 12:38 UTC
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New diagnostic tool aims to end pancreatitis misdiagnosis
Diagnosis Recruiting nowThis study is testing a new scoring system called OC26 to help doctors more accurately diagnose autoimmune pancreatitis, a condition where the immune system attacks the pancreas. Researchers will review medical data from 82 adults suspected of having the disease. If the OC26 crit…
Sponsor: Military Medical Clinical Center of the Southern Region, Ukraine • Aim: Diagnosis
Last updated Jun 27, 2026 12:39 UTC
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New scan could shine a light on hidden inflammation
Diagnosis Recruiting nowThis study is testing a new radioactive tracer called [18F]AlF-FAPI-74 for PET/CT scans to see if it can find inflammation better than the current standard tracer. The trial will involve 140 people with fever of unknown origin, IgG4-related disease, or axial spondylarthritis. Res…
Phase 2/3 • Sponsor: Universitaire Ziekenhuizen KU Leuven • Aim: Diagnosis
Last updated Jun 27, 2026 12:09 UTC
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New registry aims to unlock secrets of rare diseases
Knowledge-focused Recruiting nowThis study is creating a large database to collect information on people with rare diseases like amyloidosis, sarcoidosis, and Gaucher disease. Researchers will track patients' health over time, including their symptoms, treatments, and outcomes. The goal is to improve diagnosis …
Sponsor: Hospital Italiano de Buenos Aires • Aim: Knowledge-focused
Last updated Jun 27, 2026 08:11 UTC