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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Can a JAK inhibitor boost remission in IgG4-Related disease?
Disease control CompletedResearchers are testing whether adding tofacitinib to prednisone works better than prednisone alone for people with active IgG4-related disease. The trial enrolls 58 adults with active disease and assigns them to either tofacitinib plus prednisone or prednisone alone. The main go…
Sponsor: Peking University People's Hospital • Aim: Disease control
Last updated Sep 20, 2026 00:00 UTC
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New drug shows promise for rare inflammatory disease
Disease control CompletedThis phase 2 trial tested the drug zanubrutinib in 10 people with IgG4-related disease, a condition that causes swelling in glands and other organs. The goal was to see if the drug could reduce gland size and inflammation over 24 weeks. Researchers measured changes using PET-MRI …
Phase 2 • Sponsor: Matthew C. Baker • Aim: Disease control
Last updated Jun 27, 2026 12:02 UTC
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New drug combo shows promise for rare autoimmune disease
Disease control CompletedThis study tested a new drug called rilzabrutinib combined with steroids in 27 adults with active IgG4-related disease, a rare condition causing inflammation and organ damage. The goal was to see if the combination could prevent disease flares better than steroids alone. Particip…
Phase 2 • Sponsor: Principia Biopharma, a Sanofi Company • Aim: Disease control
Last updated Jun 27, 2026 07:54 UTC
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Scientists investigate immune cell role in rare IgG4 disease
Knowledge-focused CompletedThis study looked at a rare disease called IgG4-related disease, which causes swelling and scarring in organs. Researchers compared immune cells in the blood of 75 people: those with the disease, those with Sjogren syndrome, and healthy volunteers. The goal was to better understa…
Sponsor: Assistance Publique Hopitaux De Marseille • Aim: Knowledge-focused
Last updated Jun 27, 2026 12:31 UTC