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
62 clinical trials for this condition and its sub-types.
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Broader categories
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New drug aims to shield autoimmune patients from Life-Threatening infections
Disease control Recruiting nowThis study tests whether a medicine called Panzyga can prevent major infections in people with autoimmune or rheumatic conditions who have low antibody levels after receiving B-cell depletion therapy. About 360 adults will receive either Panzyga or a placebo, and researchers will…
Phase: PHASE3 • Sponsor: Octapharma • Aim: Disease control
Last updated Aug 14, 2026 00:00 UTC
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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: PHASE3 • 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: PHASE1 • Sponsor: Nanjing University School of Medicine • Aim: Disease control
Last updated Jul 08, 2026 00:00 UTC
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Could stem cells plus supercharged immune cells tame autoimmune disease?
Disease control Recruiting nowThis early-phase study tests whether combining mesenchymal stem cells (MSCs) with specially engineered immune cells (CAR-T cells) is safe and can help control autoimmune diseases. About 30 adults with established autoimmune conditions will receive the treatment and be monitored f…
Phase: PHASE1, PHASE2 • Sponsor: Shenzhen Geno-Immune Medical Institute • Aim: Disease control
Last updated Jun 27, 2026 14:03 UTC
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Engineered immune cells take aim at autoimmune diseases
Disease control Recruiting nowThis study tests whether specially engineered immune cells, called CAR-T cells, can safely treat autoimmune diseases. The cells are designed to target and suppress B cells, which play a key role in many autoimmune conditions. The trial involves 30 adults and focuses on safety and…
Phase: PHASE1, PHASE2 • Sponsor: Shenzhen Geno-Immune Medical Institute • Aim: Disease control
Last updated Jun 27, 2026 14:02 UTC
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New blood filter could improve treatment for autoimmune diseases
Disease control Recruiting nowThis study is testing a new blood filter called the Plasma Filter PX2. It is used in a procedure called therapeutic plasma exchange, which removes harmful substances from the blood of people with autoimmune diseases. About 46 adults will take part, and the study will check if the…
Phase: NA • Sponsor: Fresenius Medical Care Deutschland GmbH • Aim: Disease control
Last updated Jun 27, 2026 14:02 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: PHASE2 • 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…
Phase: NA • Sponsor: Peking Union Medical College Hospital • Aim: Disease control
Last updated Jun 27, 2026 12:04 UTC
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MRNA therapy takes on autoimmune diseases in early trial
Disease control Recruiting nowThis early-stage study tests a new mRNA-based treatment (ABO2203) for people with autoimmune diseases that haven't improved with standard care. The therapy works by directing the body's immune cells to attack and control overactive immune cells causing the disease. The study will…
Phase: EARLY_PHASE1 • Sponsor: Ruijin Hospital • Aim: Disease control
Last updated Jun 27, 2026 12:00 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: PHASE1, PHASE2 • Sponsor: Acepodia Biotech, Inc. • Aim: Disease control
Last updated Jun 27, 2026 09:04 UTC
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Gene therapy shot aims to tame autoimmune diseases in early trial
Disease control Recruiting nowThis 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…
Phase: EARLY_PHASE1 • Sponsor: Tongji Hospital • Aim: Disease control
Last updated Jun 27, 2026 08:11 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: PHASE2, PHASE3 • 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: PHASE2 • 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: PHASE2, PHASE3 • Sponsor: Universitaire Ziekenhuizen KU Leuven • Aim: Diagnosis
Last updated Jun 27, 2026 12:09 UTC
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Can the shingles vaccine protect those with autoimmune conditions?
Prevention Recruiting nowThis study is testing whether the shingles vaccine (RZV) works and is safe for adults with autoimmune rheumatic diseases like rheumatoid arthritis or lupus who are taking immune-suppressing drugs. Two hundred participants will receive two doses of the vaccine. Researchers will me…
Phase: PHASE4 • Sponsor: University of Sao Paulo General Hospital • Aim: Prevention
Last updated Jun 27, 2026 11:01 UTC
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20,000 volunteers needed to decode immune disease genetics
Knowledge-focused Recruiting nowThis study will analyze DNA from up to 20,000 people, including those with immune disorders and their relatives, to find genetic variants linked to conditions like atopy, autoimmunity, and primary immunodeficiency. Researchers will also study how best to return genetic results to…
Sponsor: National Institute of Allergy and Infectious Diseases (NIAID) • Aim: Knowledge-focused
Last updated Aug 18, 2026 05:00 UTC
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New registry aims to uncover risks of common transplant drug in pregnancy
Knowledge-focused Recruiting nowThis registry follows pregnant women who have taken mycophenolate, a drug used to prevent organ rejection and treat autoimmune diseases. Researchers will collect data on pregnancy complications and birth defects up to one year after birth. The goal is to better understand the dru…
Sponsor: Genentech, Inc. • Aim: Knowledge-focused
Last updated Aug 12, 2026 00:00 UTC
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Massive gene hunt aims to unlock secrets of immune disorders
Knowledge-focused Recruiting nowThis study is looking for the genes that cause certain immune system problems. Researchers want to understand why some people get sick and others don't, even in the same family. Up to 5,000 people with immune disorders and their relatives will give blood or cheek cell samples for…
Sponsor: National Institute of Allergy and Infectious Diseases (NIAID) • Aim: Knowledge-focused
Last updated Jul 17, 2026 00:00 UTC
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Protein clue could unlock new autoimmune treatments
Knowledge-focused Recruiting nowThis study is measuring a protein called STIM1 on the surface of immune cells in 670 people with various autoimmune diseases. The goal is to find which diseases have high levels of this protein, which could help researchers develop new antibody-based treatments. Participants prov…
Sponsor: University Hospital, Brest • Aim: Knowledge-focused
Last updated Jun 28, 2026 00:00 UTC
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Frozen hope: new study banks testicular tissue for boys at risk of infertility
Knowledge-focused Recruiting nowThis study collects and freezes testicular tissue from boys and young men who are at risk of becoming infertile due to cancer, autoimmune disorders, or their treatments. The tissue is stored in a research bank, with the hope that future medical advances will allow it to be used t…
Phase: NA • Sponsor: University of Pittsburgh • Aim: Knowledge-focused
Last updated Jun 27, 2026 12:23 UTC
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When to boost? new trial seeks best COVID-19 shot timing for vulnerable patients
Knowledge-focused Recruiting nowThis study looks at 320 adults who are about to start strong immune-suppressing therapy and have already had at least two COVID-19 shots. Researchers want to find out if giving a COVID-19 booster right before starting therapy or waiting six months provides better protection. Part…
Phase: PHASE3 • Sponsor: Kirby Institute • Aim: Knowledge-focused
Last updated Jun 27, 2026 09:08 UTC
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Massive new registry aims to unlock secrets of biologic drugs
Knowledge-focused Recruiting nowThis study is creating a large registry of 800 people receiving biologic medications for immune conditions like autoimmune diseases and primary immune deficiencies. Researchers will collect blood samples and health data to better understand how these treatments work and who respo…
Sponsor: Icahn School of Medicine at Mount Sinai • Aim: Knowledge-focused
Last updated Jun 27, 2026 09:05 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