[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"immunoglobulin-g4-related-disease\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:immunoglobulin-g4-related-disease":27},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,5,0,[8,41,71,127,151],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":15,"eligibilityCriteria":16,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":23,"briefSummary":25,"conditions":26,"keywords":4,"overallStatus":28,"whyStopped":4,"lastUpdateSubmitDate":29,"lastUpdatePostDateStruct":30,"startDateStruct":33,"completionDateStruct":35,"leadSponsor":37,"locationsCount":40},"100607564","phase-3-a-52-week-study-of-rilzabrutinib-efficacy-and-safety-compared-to-placebo-in-adults-diagnosed-with-igg4-related-disease-100607564",false,"NCT07190196","A 52-week Study of Rilzabrutinib Efficacy and Safety Compared to Placebo in Adults Diagnosed With IgG4-related Disease","A Randomized, Phase 3, Double-blind, 52-week Study to Evaluate the Efficacy and Safety of Rilzabrutinib (SAR444671) Compared to Placebo in Adult Participants With Active IgG4-related Disease","RILIEF","Inclusion Criteria:\n\n* Participants must have an adjudicated clinical diagnosis of IgG4-RD\n* Participants meeting Step 1 Entry criteria of 2019 ACR\u002FEULAR classification criteria for IgG4-RD and Total inclusion points are ≥20\n* Participants with active disease at screening in at least one organ system, excluding lymph nodes, as an IgG4-RD Responder Index total activity score ≥ 2\n* Participants with history or current involvement of at least 1 organ\u002Fsite (excluding lymph nodes) affected with IgG4-RD.\n* Participants with active IgG4-RD controlled for at least 2 weeks while on a stable dose of GC.\n* Participants willing to taper off GC after starting IMP.\n* Participants willing and able to participate in repeated study protocol mandated or clinically indicated imaging procedures to assess IgG4-RD such as computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography (PET), or ultrasound.\n* Participants who have an up-to-date vaccination status as per local guidelines. The last dose of live vaccines should be received at least 30 days before Day 1.\n* Contraceptive use by men and women should be consistent with local regulations regarding the methods of contraception for those participating in clinical studies.\n\nExclusion Criteria:\n\n* Meet any Step 2 Exclusion criteria from the 2019 ACR\u002FEULAR classification criteria for IgG4-RD.\n* History of retroperitoneal fibrosis, sclerosing mesenteritis, fibrosing mediastinitis, or other overwhelmingly fibrotic expression of IgG4-RD that is the sole disease manifestation.\n* Active malignancy or history of malignancy within 5 years before Day 1, except completely treated in situ carcinoma of the cervix, completely treated, and resolved nonmetastatic squamous or basal cell carcinoma of the skin.\n* Known or suspected immunodeficiency, including history of invasive opportunistic infections (eg, histoplasmosis, listeriosis, coccidioidomycosis, pneumocystosis, and aspergillosis) despite infection resolution, or otherwise recurrent infections of abnormal frequency or prolonged duration suggesting an immune compromised status, as judged by the Investigator.\n* History of serious infections with the potential for recurrence (as judged by the Investigator), with less than 4 weeks interval between resolution of serious infection and first dose of study drug, or currently active moderate to severe infection at Screening (Grade 2 or higher).\n* Current or chronic history of liver disease unrelated to IgG4-RD.\n* Refractory nausea and vomiting, malabsorption, external biliary shunt, bariatric surgery, or significant bowel resection that would preclude adequate rilzabrutinib\u002Fplacebo absorption.\n* History of solid organ transplant.\n* Planned major surgical procedure during the participation in this study.\n* History of drug abuse within the previous 12 months.\n* Alcoholism or excessive alcohol use, defined as regular consumption of more than approximately 3 standard drinks per day.\n* Prior participation in any rilzabrutinib studies or other BTK inhibitor studies.\n* History of treatment with an investigational drug within 6 months or 5 half-lives of the investigational drug, whichever is longer.\n* Laboratory abnormalities at the screening visit identified by the central laboratory The above information is not intended to contain all considerations relevant to a participant's potential participation in a clinical trial.","ALL","18 Years",{"count":20,"type":21},124,"ESTIMATED","INTERVENTIONAL",[24],"PHASE3","This is a Phase 3, parallel group, 2-arm, randomized, double blind, placebo-controlled, 52-week treatment study to assess the efficacy and safety of rilzabrutinib as a treatment for adult patients with active IgG4-RD.\n\nThe purpose of this study is to measure time to IgG4-RD clinical disease flare, and other relevant efficacy endpoints including flare-free rate, control of IgG4-RD disease activity, use of GC rescue and safety parameters such as treatment-emergent adverse events, clinical laboratory values and electrocardiograms (ECG) in participants aged 18 years and above, diagnosed with IgG4-RD and treated with rilzabrutinib tablets over a 52-week placebo-controlled period.\n\nStudy details include:\n\nThe study duration will be up to 60 weeks, including a 4 to 6-week screening period, a 52-week double blind treatment period, and 2 weeks of follow up (plus an optional OLE of 108 weeks).\n\nThe number of visits will be 16 (plus an optional 9 visits during the OLE).",[27],"Immunoglobulin G4 Related Disease","RECRUITING","2026-06-29",{"date":31,"type":32},"2026-07-01","ACTUAL",{"date":34,"type":32},"2025-09-26",{"date":36,"type":21},"2030-12-25",{"name":38,"class":39},"Sanofi","INDUSTRY",79,{"id":42,"slug":43,"hasResults":11,"nctId":44,"briefTitle":45,"officialTitle":46,"acronym":4,"eligibilityCriteria":47,"healthyVolunteers":11,"sex":17,"minAge":48,"maxAge":18,"enrollmentInfo":49,"targetDuration":4,"studyType":22,"phases":51,"briefSummary":53,"conditions":54,"keywords":56,"overallStatus":61,"whyStopped":4,"lastUpdateSubmitDate":62,"lastUpdatePostDateStruct":63,"startDateStruct":65,"completionDateStruct":67,"leadSponsor":69,"locationsCount":4},"100610051","phase-2-study-to-evaluate-the-pharmacokinetics-pharmacodynamics-safety-and-tolerability-of-inebilizumab-in-pediatric-participants-with-igg4-rd-100610051","NCT07222553","Study to Evaluate the Pharmacokinetics, Pharmacodynamics, Safety, and Tolerability of Inebilizumab in Pediatric Participants With IgG4-RD","Open-label, Uncontrolled, Multicenter Trial to Evaluate the Pharmacokinetics, Pharmacodynamics, Safety, and Tolerability of Inebilizumab in Children From 2 Years to Less Than 18 Years of Age With Immunoglobulin G4-related Disease (IgG4-RD)","Key Inclusion Criteria:\n\n1. Participants must weigh ≥ 17 kg to be eligible for enrollment.\n2. Participant has provided informed consent\u002Fassent before initiation of any study-specific activities\u002Fprocedures. Participant's legally authorized representative has provided informed consent when the participant is legally too young to provide informed consent, and the participant has provided written assent based on local regulations and\u002For guidelines before any study-specific activities\u002Fprocedures being initiated.\n3. Age 2 to \\\u003C 18 years at the time of screening. For participants who reach the age of legal consent during the clinical study, notification will be required, and a new consent form must be signed by the participant for continuation in the study.\n4. Clinical diagnosis of IgG4-RD.\n5. Fulfillment of the 2019 American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) classification criteria as determined by the principal investigator (PI) at screening. Specifically, participants must meet the classification criteria entry requirements (including involvement of one of the following organs: pancreas, bile ducts\u002Fbiliary tree, orbits, lungs, kidneys, lacrimal glands, major salivary glands, retroperitoneum, aorta, pachymeninges, or thyroid gland \\[Riedel's thyroiditis\\]), must not meet any of the classification criteria exclusions, and must achieve at least 20 classification criteria inclusion points.\n6. Receipt of all age-appropriate and locally-required vaccinations before screening.\n7. Participants requiring treatment in addition to or other than glucocorticoids (GCs) for IgG4-RD according to PI's assessment at screening.\n8. Participants who are on GCs for the treatment of IgG4-RD should remain on a stable dose for at least 2 weeks before enrollment (Day 1). Tapering post enrollment will be at PI's discretion.\n\nKey Exclusion Criteria:\n\n1. Participants with any of the following abnormal liver function tests in the presence of hepatobiliary IgG4-RD activity:\n\n   * aspartate aminotransferase (AST) \\> 10 × upper limit of normal (ULN)\n   * alanine aminotransferase (ALT) \\> 10 × ULN\n   * total bilirubin (TBL) \\> 5 × ULN Screening liver function tests may be repeated before Day 1 to permit abnormal values due to hepatobiliary IgG4-RD activity to respond to GC treatment.\n2. Evidence of significant hepatic, renal, or metabolic dysfunction or significant hematological abnormality, including any of the following at screening (one repeat test may be conducted to confirm results within the same screening period):\n\n   * platelet count \\\u003C 75000\u002FμL (or \\\u003C 75 × 109\u002FL)\n   * absolute neutrophil count \\\u003C 1200 cells\u002FμL\n   * total Ig \\\u003C 600 mg\u002FdL\n   * CD4 T lymphocyte count \\\u003C 300 cells\u002FµL\n   * hemoglobin \\\u003C 8 g\u002FdL (or \\\u003C 80 g\u002FL).\n3. Estimated glomerular filtration rate \\\u003C 45 mL\u002Fmin\u002F1.73 m\\^2.\n4. B-cell counts \\\u003C one-half of the lower limit of normal (LLN) for age according to the central laboratory.\n5. Diagnosed with a concurrent autoimmune disease that is uncontrolled or requires any prohibited medication (unless approved by the medical monitor).\n6. Clinically significant serious active or chronic viral, bacterial, or fungal infection that requires treatment with anti-infectives, hospitalization, or, in the investigator's opinion, represents an additional risk to the participant, within 2 months before Day 1 of study.\n7. Known history of congenital or acquired immunodeficiency (eg, due to human immunodeficiency virus \\[HIV\\] infection, splenectomy, immunosuppression-related or idiopathic T-cell deficiencies) that predisposes the participant to infection.\n8. Positive test for chronic hepatitis B infection at screening, defined as either: (1) Positive hepatitis B surface antigen (HBsAg); or (2) Positive hepatitis B core antibody (anti-HBc) PLUS negative hepatitis B surface antibody (anti-HBs). Note: Participants with a positive anti-HBs only, or a positive anti-HBc plus positive anti-HBs and negative HBsAg, are eligible to enroll.\n9. Receipt of any of the following before Day 1: alemtuzumab, total lymphoid irradiation, bone marrow transplant, T-cell vaccination therapy.\n10. Receipt of any of the following within 2 months before Day 1: azathioprine, mycophenolate mofetil, cyclosporine, methotrexate, cyclophosphamide, tocilizumab, satralizumab, eculizumab, and mitoxantrone.\n11. Receipt of rituximab or any experimental B-cell depleting agent (eg, ocrelizumab, obinutuzumab, ofatumumab, inebilizumab), or any non-depleting B-cell-directed therapy (eg, belimumab), abatacept, within 6 months before screening unless B-cell counts have returned to ≥ one-half the LLN.\n12. Receipt of any live or attenuated vaccine (administration of inactivated \\[killed\\] vaccine is acceptable) within 4 weeks before Day 1, Bacillus Calmette-Guérin vaccine within 1 year of screening, or blood transfusion within 4 weeks before screening or during screening.","2 Years",{"count":50,"type":21},15,[52],"PHASE2","The primary objectives of this study are to characterize the pharmacokinetics (PK) and pharmacodynamics (PD), as well as to assess the safety and tolerability, of inebilizumab in pediatric participants with IgG4-RD.",[55],"Immunoglobulin G4-related Disease",[55,57,58,59,60],"Inebilizumab","AMG 335","IgG4-related disease","IgG4-RD","NOT_YET_RECRUITING","2026-05-01",{"date":64,"type":32},"2026-05-04",{"date":66,"type":21},"2026-07-31",{"date":68,"type":21},"2031-06-10",{"name":70,"class":39},"Amgen",{"id":72,"slug":73,"hasResults":11,"nctId":74,"briefTitle":75,"officialTitle":76,"acronym":4,"eligibilityCriteria":77,"healthyVolunteers":11,"sex":17,"minAge":4,"maxAge":4,"enrollmentInfo":78,"targetDuration":80,"studyType":81,"phases":4,"briefSummary":82,"conditions":83,"keywords":110,"overallStatus":28,"whyStopped":4,"lastUpdateSubmitDate":116,"lastUpdatePostDateStruct":117,"startDateStruct":119,"completionDateStruct":121,"leadSponsor":123,"locationsCount":126},"100560175","institutional-registry-of-rare-diseases-100560175","NCT06573723","Institutional Registry of Rare Diseases","Institutional Registries of Rare Diseases at Hospital Italiano de Buenos Aires (HIBA)","Inclusion Criteria:\n\n* Clinical and\u002For molecular diagnosis of any of the following rare diseases: Amyloidosis, Sarcoidosis, Phacomatosis, Pheochromocytoma, Paraganglioma, Von Hippel-Lindau Disease, Immunoglobulin G4-Related Disease, Demyelinating Diseases, Inborn Errors of Metabolism, Eosinophilic Gastrointestinal Disorders, Hypertrophic Cardiomyopathy, Gaucher Disease, Congenital Adrenal Hyperplasia, Hereditary Angioedema, Pulmonary Hypertension, Wilson Disease, Vascular Anomalies, Mastocytosis, Multiple Endocrine Neoplasia, Inflammatory Bowel Diseases, Prader-Willi Syndrome, Hirschsprung Disease, or Cushing Syndrome.\n* Must be followed at Hospital Italiano de Buenos Aires.\n\nExclusion Criteria:\n\n\\- Refusal to participate in the study or in the informed consent process.",{"count":79,"type":21},380,"10 Years","OBSERVATIONAL","The goal of this observational study is to create a single macro registry system with data collection on common clinical features, grouping the different rare diseases (RD).\n\nMoreover, the specific goals are to generate an alert system for possible cases of RD with data from the electronic medical record, to describe the occurrence of RD in the evaluated population, to characterize the population, to describe patterns of diagnosis and treatment of RD present at the time, and to explore patient-reported outcomes.",[84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,102,103,104,105,106,107,108,109],"Rare Diseases","Amyloidosis","Sarcoidosis","Phacomatosis","Pheochromocytoma","Paraganglioma","Von Hippel-Lindau Disease","Immunoglobulin G4-Related Disease","Demyelinating Diseases","Inborn Errors of Metabolism","Eosinophilic Gastrointestinal Disorders","Hypertrophic Cardiomyopathy","Gaucher Disease","Congenital Adrenal Hyperplasia","Hereditary Angioedema","Pulmonary Hypertension","Wilson Disease","Vascular Anomalies","Mastocytosis","Multiple Endocrine Neoplasia","Inflammatory Bowel Diseases","Prader-Willi Syndrome","Hirschsprung Disease","Cushing Syndrome","HHT","Hemorrhagic Hereditary Telangiectasia",[111,112,113,87,114,115,90,91,92,93,94,95,96,97,98,99,100,101,102,103,104,105,106,107,109],"rare diseases","amyloidosis","sarcoidosis","pheochromocytoma","paraganglioma","2026-01-12",{"date":118,"type":32},"2026-01-14",{"date":120,"type":32},"2024-07-01",{"date":122,"type":21},"2034-12-31",{"name":124,"class":125},"Hospital Italiano de Buenos Aires","OTHER",1,{"id":128,"slug":129,"hasResults":11,"nctId":130,"briefTitle":131,"officialTitle":132,"acronym":133,"eligibilityCriteria":134,"healthyVolunteers":135,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":136,"targetDuration":4,"studyType":22,"phases":138,"briefSummary":140,"conditions":141,"keywords":4,"overallStatus":61,"whyStopped":4,"lastUpdateSubmitDate":142,"lastUpdatePostDateStruct":143,"startDateStruct":145,"completionDateStruct":147,"leadSponsor":149,"locationsCount":4},"100536876","study-of-the-c5ac5ar1-axis-in-igg4-associated-disease-a-potential-therapeutic-target-100536876","NCT06270524","Study of the C5a\u002FC5aR1 Axis in IgG4-associated Disease: a Potential Therapeutic Target","Pilot Study of the C5a\u002FC5aR1 Axis in IgG4-associated Disease: a Potential Therapeutic Target. C5-MAG4 Study.","C5-MAG4","Inclusion Criteria for patients suffering from IG4-related disease and patients in remission :\n\n* age \\> 18\n* diagnosis of IgG4-associated disease according to ACR\u002FEULAR 2019 classification criteria (4,5) with a score ≥20.\n* disease activity score (IgG4-RD Responder Index) ≥2 in at least one affected organ\n\nExclusion Criteria for patients suffering from IG4-related disease and patients in remission :\n\n* presence of a differential diagnosis (\"IgG4-RD mimicker\") or exclusion criterion according to ACR\u002FEULAR 2019 classification criteria (4,5).\n* systemic corticosteroid treatment ≥5 mg\u002Fday ongoing or \\\u003C 1 month ; Study C5-MAG4\\_RCAPHM21\\_0433 Protocol Version 1.1 of 16\u002F01\u002F2024 Page 15 of 41\n* immunosuppressive therapy ongoing or \\\u003C 3 months ;\n* current biotherapy treatment or \\\u003C 6 months;\n* absence of signed informed consent;\n* absence of affiliation to a Social Security scheme.\n* patient participating in an ongoing therapeutic trial ;\n* any condition which, in the opinion of the investigator, could influence the results of the study.\n\nInclusion criteria for healthy volunteers :\n\n\\- age \\> 18\n\nExclusion criteria for healthy volunteers :\n\n* infectious episode (temperature \\>38°C, flu-like signs) in the 30 days prior to inclusion;\n* known inflammatory (including IG4-related disease) or autoimmune pathology;\n* treatment with corticosteroids in progress or taken in the month prior to sampling, or immunosuppressants in progress or taken in the 3 months prior to sampling, or biotherapy in the 6 months prior to sampling;\n* absence of signed informed consent;\n* absence of affiliation to a Social Security scheme;\n* persons taking part in an ongoing therapeutic trial;\n* any condition which, in the opinion of the investigator, could influence the results of the study.",true,{"count":137,"type":21},66,[139],"NA","This is a monocentric, comparative, cross-sectional, case-control study seeking to identify abnormalities of the C5a-C5aR1 axis between a population of patients with active IgG4-associated disease (MAG4) and two control groups: healthy subjects without MAG4 and patients with MAG4 in remission.",[91],"2024-06-25",{"date":144,"type":32},"2024-06-26",{"date":146,"type":21},"2024-09-01",{"date":148,"type":21},"2027-04-30",{"name":150,"class":125},"Assistance Publique Hopitaux De Marseille",{"id":152,"slug":153,"hasResults":11,"nctId":154,"briefTitle":155,"officialTitle":156,"acronym":4,"eligibilityCriteria":157,"healthyVolunteers":135,"sex":17,"minAge":18,"maxAge":158,"enrollmentInfo":159,"targetDuration":161,"studyType":81,"phases":4,"briefSummary":162,"conditions":163,"keywords":4,"overallStatus":28,"whyStopped":4,"lastUpdateSubmitDate":167,"lastUpdatePostDateStruct":168,"startDateStruct":170,"completionDateStruct":172,"leadSponsor":174,"locationsCount":126},"100491657","immunoglobulin-g4-and-immunoglobulin-e-antibodies-in-a-population-with-adverse-reactions-to-foodstuffs-related-symptoms-100491657","NCT05681975","Immunoglobulin G4 and Immunoglobulin E Antibodies in a Population With Adverse Reactions to Foodstuffs-related Symptoms","Food-specific Immunoglobulin G4 and Immunoglobulin E Antibody Reactions in a Population With Adverse Reactions to Foodstuffs-related Symptoms","Inclusion Criteria:\n\n* Adults (\\>18 years of age)\n* To present symptoms related to adverse reactions to foodstuffs (ARFS)\n\nExclusion Criteria:\n\n* Antibiotic treatment","80 Years",{"count":160,"type":21},200,"5 Years","The aim of this study is to analyze a population with symptoms associated with adverse reactions to foodstuffs (ARFS). To determine the levels of food-specific immunoglobulin G4 (IgG4) and immunoglobulin E (IgE) antibody reactions (AbR).",[164,91,165,166],"Immunoglobulin E-Mediated Food Allergy","Symptoms and Signs","Adverse Reaction to Food","2023-01-03",{"date":169,"type":32},"2023-01-12",{"date":171,"type":32},"2017-10-01",{"date":173,"type":21},"2027-12",{"name":175,"class":125},"Universidad Politecnica de Madrid"]