[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"igg4-related-disease\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:igg4-related-disease":55},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,15,0,[8,40,69,95,124,151,172,194,221,244,266,281,305,332,352],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":4,"eligibilityCriteria":15,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":18,"targetDuration":4,"studyType":21,"phases":22,"briefSummary":25,"conditions":26,"keywords":4,"overallStatus":28,"whyStopped":4,"lastUpdateSubmitDate":29,"lastUpdatePostDateStruct":30,"startDateStruct":33,"completionDateStruct":35,"leadSponsor":37,"locationsCount":4},"100638579","phase-2-a-study-of-imm0306-in-igg4-related-disease-100638579",false,"NCT07621939","A Study of IMM0306 in IgG4-Related Disease","A Phase II\u002FIII Clinical Study to Evaluate the Efficacy and Safety of IMM0306 in Participants With IgG4-Related Disease (IgG4-RD)","Inclusion Criteria:\n\n* Clinical diagnosis of IgG4-RD;\n* Meeting 2019 ACR\u002FEULAR classification criteria with an inclusion score ≥20;\n* At least two organs\u002Fsites involved during the disease course;\n* Prior or recent IgG4-RD flare requiring initiation or continuation of GC treatment at informed consent.\n\nExclusion Criteria:\n\n* Fibrotic manifestation as the only clinical manifestation of the current relapse;\n* Significant hematologic or hepatic abnormalities;\n* Recent B-cell-depleting therapy, alkylating agents, DMARDs or immunosuppressants;\n* Other chronic active immune diseases requiring long-term use of immunosuppressants;\n* Active malignancy or active malignancy within 10 years;\n* Significant cardiac disease;\n* Active infection or active TB;\n* Severe pulmonary disease;\n* Recent opportunistic infection;\n* Alcohol\u002Fdrug abuse;\n* Pregnancy, lactation or failure to meet contraception requirements.","ALL","18 Years",{"count":19,"type":20},125,"ESTIMATED","INTERVENTIONAL",[23,24],"PHASE2","PHASE3","The goal of this clinical trial is to learn if IMM0306 works to reduce the risk of disease relapse in participants with IgG4-related disease (IgG4-RD). It will also learn about the safety and tolerability of IMM0306. The main questions it aims to answer are:\n\n* Does IMM0306 reduce the risk of disease relapse in participants with IgG4-RD?\n* What medical problems do participants have when receiving IMM0306?\n* How does IMM0306 behave in the body, and does the body develop anti-drug antibodies against IMM0306? In the Phase II part, all participants will receive IMM0306. In the Phase III part, researchers will compare IMM0306 with placebo to evaluate whether IMM0306 reduces the risk of disease relapse in participants with IgG4-RD.\n\nParticipants will:\n\n* Receive IMM0306 or placebo by intravenous infusion once weekly for 4 consecutive weeks, with the same treatment repeated 6 months later\n* Start a planned glucocorticoid taper from Day 1 and gradually reduce oral prednisone or equivalent until complete discontinuation after 8 weeks\n* Visit the study site for efficacy and safety assessments, including disease relapse assessment, IgG4-RD responder index assessment, laboratory tests, imaging examinations, electrocardiograms, pharmacokinetic sampling, immunogenicity sampling, biomarker sampling, and adverse event assessment.",[27],"IgG4-Related Disease","NOT_YET_RECRUITING","2026-06-01",{"date":31,"type":32},"2026-06-02","ACTUAL",{"date":34,"type":20},"2026-07",{"date":36,"type":20},"2030-05",{"name":38,"class":39},"ImmuneOnco Biopharmaceuticals (Shanghai) Inc.","OTHER",{"id":41,"slug":42,"hasResults":11,"nctId":43,"briefTitle":44,"officialTitle":44,"acronym":45,"eligibilityCriteria":46,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":47,"enrollmentInfo":48,"targetDuration":4,"studyType":21,"phases":50,"briefSummary":51,"conditions":52,"keywords":56,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":59,"lastUpdatePostDateStruct":60,"startDateStruct":62,"completionDateStruct":64,"leadSponsor":66,"locationsCount":68},"100538031","phase-2-drug-rediscovery-for-rare-immune-mediated-inflammatory-diseases-100538031","NCT06285539","Drug Rediscovery for Rare Immune Mediated Inflammatory Diseases","DRIMID","Inclusion Criteria:\n\n* Age 18 years of older\n* One of the following rare IMIDs:\n\n  * Diagnosis of Behçet's disease without refractory life, organ or sight-threatening symptoms with active disease, defined as a BDCAF \\>2 (new BDCAF) or \\>15 (old BDCAF) or with active disease, based on clinical grounds (e.g. the need to start new or additional medication\n  * Diagnosis of idiopathic inflammatory myopathy, according to diagnostic criteria:\n\nDermatomyositis: Dermatomyositis Classification Criteria according to the European Neuromuscular Centre guidelines 201852 or anti-synthetase syndrome: Anti- synthetase syndrome Classification Criteria according to the European Neuromuscular Centre guidelines 200353, both with active disease, defined as a CDASI score of ≥5 or abnormal levels of at least 1 of the following enzymes: creatine kinase (≥ 4× upper limit of normal \\[ULN\\]), aldolase (≥4 × ULN), lactate dehydrogenase (LDH ≥4 × ULN), aspartate transaminase (AST ≥4 × ULN), alanine aminotransferase (ALT ≥4 × ULN) or a MRI within the last 3 months indicative of active inflammation (e.g. edema signal pattern in affected proximal muscles) or active disease based on clinical grounds, e.g. the need to start new or additional medication\n\n* Diagnosis of IgG4-related disease, according to 2019 ACR\u002FEULAR guidelines, with active disease, defined as: IgG4-related disease responder index \\>10 or active disease based on clinical grounds, e.g. the need to start new or additional medication\n\n  * Refractory disease, defined as symptomatic disease that persists despite a 12-week trial of glucocorticoid therapy as well as lack of response to at least one other immunosuppressive agent such as methotrexate (MTX), mycophenolate mofetil (MMF), azathioprine (AZA) or rituximab or intolerance to standard-of-care treatment, as defined by the treating physician.\n  * No evidence of active or latent or inadequately treated infection with mycobacterium tuberculosis (TB) as defined by all of the following: both a negative QuantiFERON-TB Gold (QFT-G) In-Tube test and a Mantoux tuberculin skin test performed at or within 3 months prior to screening and no signs suggestive of active TB infection as determined (and documented) by a qualified radiologist or pulmonologist as per local standard of care on a chest radiograph and no history of either untreated or inadequately treated latent or active TB infection.\n\nExclusion Criteria:\n\n* Age \\\u003C18 years\n* Age ≥65 years\n* Life expectancy less than 6 months\n* Juvenile DM, myositis overlapping with other autoimmune diseases, immune mediated necrotizing myopathy (IMNM), inclusion-body myositis or cancer-associated myositis\n* End-stage IIM wherein muscle weakness is most likely due to muscle damage, rather than myositis disease activity\n* Increased risk of major cardiovascular problems\n* Current smoker or smoked for a long time in the past\n* Pregnancy or lactation\n* Previous use of other JAK inhibitors\n* Use of any investigational drug within one month prior to screening or within five half-lives of the investigational agent, whichever is longer.\n* Human Immunodeficiency Virus (HIV) infection\n* Presence of an active infection or viral hepatitis type B or C\n* History of shingles or recurrent herpes simplex infection\n* Concomitant malignancies or previous malignancies within the last five years (with exception of adequately treated basal or squamous cell carcinoma of the skin)\n* Increased risk of cancer\n* Kidney injury with estimated glomerular filtration rate \\\u003C15mL\u002Fmin\u002F1.73m2\n* Liver failure Child Pugh C\n* Absolute neutrophil count \\\u003C1\\*109\n* Absolute leukocyte count \\\u003C0.5\\*109\n* Hemoglobin \\\u003C5mmol\u002FL - Inability to comply with study and\u002For follow-up procedures\n* Known recent substance abuse (drugs or alcohol).\n* Poor tolerability of venipuncture or lack of adequate venous access for required blood sampling during the study period.\n* Previous non-adherence to immunosuppressants\n* Hypersensitivity to the active substance or to any of the excipients\n* Rare hereditary problems of galactose intolerance, total lactase deficiency or glucose-galactose malabsorption","65 Years",{"count":49,"type":20},60,[23],"Research into novel therapies for rare, immune-mediated inflammatory diseases (IMIDs) is limited due to small patient populations. Patients with Behçet's disease (BD), idiopathic inflammatory myopathy (IIM, also known as myositis) and IgG4-related disease (IgG4-RD) are treated with high-dosed glucocorticoids, methotrexate, azathioprine and mycophenolate mofetil, mostly for long periods of time with attendant risks of long-term toxicity, including infections. Therefore, there is an urgent need for new, more specific anti-inflammatory therapies such as targeted synthetic and biological disease-modifying antirheumatic drugs. Due to the role of type 1 interferon in both BD, IIM and IgG4-RD, JAK-STAT inhibition may be a promising treatment strategy in these conditions, because JAK1 is critical for the signal transduction of pro-inflammatory cytokine receptors. Previous research showed that JAK1 inhibition reduces activation of type 1 interferon-regulated proteins and key chemokines that control tissue inflammation.",[53,54,55],"Behcet's Disease","Idiopathic Inflammatory Myopathies","IgG4-related Disease",[57],"JAK-inhibition","RECRUITING","2026-05-07",{"date":61,"type":32},"2026-05-12",{"date":63,"type":32},"2024-03-12",{"date":65,"type":20},"2027-12",{"name":67,"class":39},"UMC Utrecht",6,{"id":70,"slug":71,"hasResults":11,"nctId":72,"briefTitle":73,"officialTitle":74,"acronym":4,"eligibilityCriteria":75,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":76,"targetDuration":4,"studyType":21,"phases":78,"briefSummary":79,"conditions":80,"keywords":4,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":85,"lastUpdatePostDateStruct":86,"startDateStruct":88,"completionDateStruct":90,"leadSponsor":92,"locationsCount":94},"100634120","phase-2-the-investigators-will-evaluate-the-diagnostic-performance-of-18f-alf-fapi-74-petct-in-inflammatory-disorders-and-compare-it-with-the-current-gold-standard-for-inflammation-fdg-petct-in-three-patient-cohort-patients-presenting-with-fever-of-unknown-origin-igg4-rd-and-axspa-100634120","NCT07535554","The Investigators Will Evaluate the Diagnostic Performance of [18F]-AlF-FAPI-74 PET\u002FCT in Inflammatory Disorders and Compare it With the Current Gold Standard for Inflammation, FDG PET\u002FCT, in Three Patient Cohort: Patients Presenting With Fever of Unknown Origin, IgG4-RD and AxSpA.","Prospective Diagnostic Performance of PET\u002FCT Using the Novel Fibroblast Imaging Tracer [18F]-AlF-FAPI-74 Versus Standard of Care [18F]-FDG in Inflammatory Disorders","Inclusion Criteria FUO:\n\n* An illness of more than 3 weeks' duration,\n* Temperature exceeding 38.3°C on \\> 3 occasions, or elevated inflammatory markers on \\> 3 occasions\n* Diagnosis uncertain despite appropriate first-line investigations.\n\nInclusion criteria IgG4-RD:\n\n* High clinical suspicion of IgG4-RD by an experienced clinician of Internal Medicine, based on anamnesis, physical examination, first-line investigations and blood tests.\n* Patients with a new diagnosis of IgG4-RD on histopathology and need for further assessment of disease extent with PET\u002FCT,\n* Relapse of diagnosed IgG4-RD on histopathology according to an experienced clinician of Internal Medicine\n\nInclusion criteria AxSpA:\n\n* High clinical suspicion of axial spondyloarthropahy according to an experienced rheumatology (based on inflammary back pain \\> 3 months, insidious onset, morning stifness, improvement with exercise and worsening in rest, pain worse at night, age at onset \\\u003C45 years of age, imaging findings).\n* Patient has persisting inflammatory back pain after 2 different types of NSAID's (tried for over 2-4 weeks), and is therefore eligible for biological DMARDS therapy.\n\nExclusion Criteria:\n\n* Participant is mentally or legally incapacitated, doesn't understand the study design or is not willing or capable to undergo all study-specific procedures.\n* Any disorder or condition, which in the Investigator's opinion might jeopardise the participant's safety or compliance with the protocol.\n* Any prior or concomitant treatment(s) that might jeopardise the participant's safety or that would compromise the integrity of the Trial.\n* Female who is pregnant (urinary hCG test will be performed in every WOCBP), breast-feeding or intends to become pregnant or is of child-bearing potential and not using an adequate, highly effective contraceptive (with a relatively high Pearl Index: natural methods, minipill outside postpartum period, spermicides or condoms in monotherapy or no usage of contraception when sexually active are not accepted).\n* Participation in an interventional Trial with an investigational medicinal product (IMP) or device when the trial designs are not considered compatible by the study team.\n* Participation in a clinical scientific study in the last 12 months with a radiation exposure caused by the experimental procedures greater than 1 mSv.\n* Participant has a known hypersensitivity to \\[18F\\]AlF-FAPI-74 or the used excipients.\n* Active treatment has already commenced",{"count":77,"type":20},140,[23,24],"The aim of the study to evaluate the performance of new \\[18F\\]-AlF-FAPI-74 PET\u002FCT in three inflammatory disorders (fever of unknown origin, IgG4-related disease and axial spondyloarthritis) and compare with the current stand-of-care \\[18F\\]-FDG PET\u002FCT",[81,82,83,84],"Fever of Unknown Origin","IgG4 Related Disease","Axial Spondylarthritis (axSpA)","Inflammation of Unknown Origin","2026-04-14",{"date":87,"type":32},"2026-04-17",{"date":89,"type":32},"2025-09-17",{"date":91,"type":20},"2030-03-31",{"name":93,"class":39},"Universitaire Ziekenhuizen KU Leuven",1,{"id":96,"slug":97,"hasResults":11,"nctId":98,"briefTitle":99,"officialTitle":100,"acronym":4,"eligibilityCriteria":101,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":102,"enrollmentInfo":103,"targetDuration":4,"studyType":21,"phases":105,"briefSummary":107,"conditions":108,"keywords":109,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":113,"lastUpdatePostDateStruct":114,"startDateStruct":116,"completionDateStruct":118,"leadSponsor":120,"locationsCount":123},"100597703","phase-1-study-to-assess-safety-efficacy-and-persistence-of-ace1831-in-subjects-with-igg4-related-disease-100597703","NCT07061938","Study to Assess Safety, Efficacy and Persistence of ACE1831, in Subjects With IgG4-Related Disease","Phase 1b\u002F2a Prospective, Open Label, Multicenter, Single Arm Study to Assess Safety, Efficacy and Persistence of ACE1831, in Subjects With Immunoglobulin G4-Related Disease","Inclusion Criteria: To be eligible for this study, all of the following inclusion criteria must be met:\n\n* Signed Informed Consent\n* Male or female ≥ 18 to 75 years of age\n* Active IgG4-RD flare at screening with IgG4-RD Responder Index at least 2, confirmed by symptoms, labs, and\u002For imaging.\n* History of IgG4-RD involving at least 2 organs\u002Fsites, and current flare involves at least 1 organ\u002Fsite (excluding lymph nodes) requiring treatment.\n* Elevated serum IgG4 above the upper limit of normal at screening.\n* Able to receive glucocorticoids for current flare and taper to 0 mg by Day -5.\n* Contraception agreement per protocol from screening through 24 weeks after last ACE1831 dose (no LDC) or 12 months after last LDC dose (with LDC).\n* For sites in China only: prior treatment failure to glucocorticoids and at least one immunosuppressive agent.\n\nExclusion Criteria: An individual who meets any of the following criteria will be excluded from participation in this trial.\n\n* Significant conditions that impair ability to receive study treatment or comply.\n* Predominant fibrosis in affected organs.\n* Active\u002Flatent infection that would interfere with therapy (including HBV, HCV, HIV, TB, syphilis) or significant recent infection per protocol.\n* Known immunodeficiency state.\n* NYHA class III\u002FIV heart disease.\n* Severe allergy\u002Fhypersensitivity to monoclonal antibodies or relevant study agents.\n* Malignancy within 5 years (protocol exceptions apply).\n* Recent investigational agent exposure.\n* Recent B-cell depleting therapy (anti-CD20\u002Fanti-CD19) unless reconstitution per protocol.\n* Live\u002Fattenuated vaccine within 2 months.\n* Pregnant or breastfeeding.\n* Inadequate organ function\u002Fblood counts per protocol.","75 Years",{"count":104,"type":20},30,[106,23],"PHASE1","ACE1831 is an off-the-shelf, allogeneic gamma delta T (gdT) cell therapy derived from healthy donors, that is under investigation for the treatment in subjects with Immunoglobulin G4 Related Disease (IgG4-RD)",[82],[82,110,111,112],"cell therapy","IgG4-RD","ACE1831","2026-02-12",{"date":115,"type":32},"2026-02-13",{"date":117,"type":32},"2026-01-01",{"date":119,"type":20},"2027-06-20",{"name":121,"class":122},"Acepodia Biotech, Inc.","INDUSTRY",3,{"id":125,"slug":126,"hasResults":11,"nctId":127,"briefTitle":128,"officialTitle":129,"acronym":4,"eligibilityCriteria":130,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":131,"enrollmentInfo":132,"targetDuration":4,"studyType":21,"phases":134,"briefSummary":135,"conditions":136,"keywords":137,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":142,"lastUpdatePostDateStruct":143,"startDateStruct":145,"completionDateStruct":147,"leadSponsor":149,"locationsCount":94},"100594890","phase-2-a-study-of-efgartigimod-in-patients-with-igg4-related-disease-100594890","NCT07025330","A Study of Efgartigimod in Patients With IgG4-Related Disease","A Phase IIa, Single-Site, Open-Label Study of Efgartigimod in Patients With IgG4-Related Disease","Key Inclusion Criteria:\n\n* Have a clinical diagnosis of IgG4-related disease that requires treatment in the opinion of the investigator\n* Meet the 2019 ACR\u002FEULAR Classification Criteria for IgG4-Related Disease\n* Have a serum IgG4 concentration greater than or equal to 2 times the upper limit of normal at Screening\n* Have involvement of the lacrimal gland(s), salivary gland(s), and\u002For pancreas\n\n  * If lacrimal and\u002For salivary glands are involved, it must be symptomatic, including but not limited to discomfort, pain, dryness, headache, or vision changes\n  * If the pancreas is involved, it must be asymptomatic, diffuse enlargement without signs or symptoms of obstruction or evidence of major organ dysfunction in the opinion of the investigator\n* Have a prior inadequate response to, or intolerance of, glucocorticoids, or who have experienced recurrent symptoms after previous treatment with glucocorticoids\n* Are not receiving current treatment with immunosuppressive medications\n* All women must test negative for pregnancy and agree to use a reliable method of birth control\n\nKey Exclusion Criteria:\n\n* Any exclusion criteria listed in the 2019 ACR\u002FEULAR Classification Criteria for IgG4-Related Disease\n* Prior treatment with an FcRn inhibitor\n* Have conventional synthetic disease-modifying antirheumatic drug (csDMARD) or immunosuppressive use as follows:\n\n  * Treatment with glucocorticoids within 28 days prior to Baseline or planned treatment during the study\n  * Treatment with csDMARDs including but not limited to hydroxychloroquine, methotrexate, leflunomide, or sulfasalazine within 28 days prior to Baseline or planned treatment during the study\n  * Treatment with cytotoxic or immunosuppressive drugs including but not limited to cyclophosphamide, mycophenolic acid, azathioprine, cyclosporine, sirolimus, or tacrolimus within 28 days prior to Baseline or planned treatment during the study\n  * Treatment with a janus kinase (JAK) inhibitor including but not limited to tofacitinib, baricitinib, upadacitinib, or filgotinib within 28 days prior to Baseline or planned treatment during the study\n  * Treatment with a Bruton's tyrosine kinase (BTK) inhibitor including but not limited to ibrutinib, zanubrutinib, acalabrutinib, pirtobrutinib, or rilzabrutinib within 28 days prior to Baseline or planned treatment during the study\n* Have biologic disease-modifying antirheumatic drug (bDMARD) use as follows:\n\n  * Treatment with etanercept, adalimumab, or anakinra within 28 days before Baseline or planned treatment during the study\n  * Treatment with infliximab, certolizumab pegol, golimumab, abatacept, or tocilizumab within 56 days before Baseline or planned treatment during the study\n  * Treatment with a B cell depleting agent including but not limited to rituximab, ocrelizumab, obinutuzumab, ofatumumab, inebilizumab, ianalumab, or obexelimab ≤ 6 months prior to Baseline\n  * Patients who received B-cell targeted therapy \\> 6 and ≤ 12 months prior to Baseline must have a B-cell count that is within the laboratory reference range at Screening\n  * Treatment with a BAFF antagonist including but not limited to belimumab or tabalumab within 6 months before Baseline or planned treatment during the study\n  * Treatment with an IL-17 antagonist including but not limited to secukinumab, ixekizumab, or brodalumab within 6 months before Baseline or planned treatment during the study\n  * Prior treatment with other bDMARDs may be allowed at the discretion of the investigator\n* A history of, or current, inflammatory or autoimmune disease (that could affect the interpretation of safety or efficacy outcomes) other than IgG4-related disease\n* Evidence of active tuberculosis, HIV, or hepatitis B or C infection\n* History of cancer except for skin basal or squamous cell carcinoma, cervical dysplasia or carcinoma in situ that has been treated and is considered cured \\> 1 year prior to Baseline, prostate cancer considered cured for \\> 5 years with a normal prostate specific antigen, or colon cancer considered cured \\> 5 years","90 Years",{"count":133,"type":20},5,[23],"The goal of this clinical trial is to learn if efgartigimod can treat IgG4-related disease in adults. The main questions it aims to answer are:\n\nIn patients with IgG4-related disease, does treatment with efgartigimod reduce the volume of the:\n\n* lacrimal gland(s) and\u002For\n* salivary gland(s) and\u002For\n* pancreas\n\nParticipants will:\n\n* Receive efgartigimod once weekly for up to 12 weeks\n* Visit the clinic every one to six weeks for checkups and tests\n* Be asked to complete questionnaires to see how they feel on efgartigimod",[55],[138,111,139,140,141],"efgartigimod","IgG4-related disease","FcRn","FcRn inhibitor","2026-02-06",{"date":144,"type":32},"2026-02-10",{"date":146,"type":32},"2025-11-12",{"date":148,"type":20},"2028-06-01",{"name":150,"class":39},"Stanford University",{"id":152,"slug":153,"hasResults":11,"nctId":154,"briefTitle":155,"officialTitle":156,"acronym":111,"eligibilityCriteria":157,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":102,"enrollmentInfo":158,"targetDuration":4,"studyType":21,"phases":160,"briefSummary":161,"conditions":162,"keywords":4,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":163,"lastUpdatePostDateStruct":164,"startDateStruct":166,"completionDateStruct":168,"leadSponsor":170,"locationsCount":94},"100598182","phase-2-a-randomized-controlled-study-of-the-efficacy-and-safety-of-lenalidomide-in-the-treatment-of-active-igg4-related-disease-100598182","NCT07068165","A Randomized Controlled Study of the Efficacy and Safety of Lenalidomide in the Treatment of Active IgG4-related Disease","A Randomized Controlled Study of the Efficacy and Safety of Lenalidomide in the Treatment of Active IgG4-RD","Inclusion Criteria:\n\n1. Patients aged between 18 and 75 years who meet the 2019 American College of Rheumatology\u002FEuropean League Against Rheumatism (ACR\u002FEULAR) classification criteria for IgG4-RD；\n2. Patients with active disease: For patients with initial treatment or relapse after drug withdrawal, active disease is defined as having an IgG4-RD Response Index (RI) of at least 2 points in at least one organ at the time of screening; for those experiencing relapse while on treatment, active disease is defined as having an IgG4-RD RI of at least 3 points in at least one organ at the time of screening；\n3. Patients with the clinical subtype of proliferative IgG4-RD；\n4. Patients who have no plans for pregnancy within the next 18 months and who agree to use reliable contraceptive measures during the study period；\n\nExclusion Criteria:\n\n1. IgG4-RD patients with only fibrotic features;\n2. Absolute neutrophil count \\\u003C1.5×10\\^9 \u002FL or platelet count \\\u003C100×10\\^9\u002FL;\n3. Creatinine clearance less than 60 ml\u002F min;\n4. Liver function Child-Pugh grade B or above;\n5. Chronic active infection requiring systemic treatment;\n6. Diagnosed with malignant tumor in the past five years;\n7. Patients with a history of thrombosis;\n8. Using biological agents within six months;\n9. Known to be allergic to lenalidomide or thalidomide;\n10. Pregnant or lactating women;\n11. Patients with any other medical conditions or for specific reasons judged by the investigator to be ineligible to participate in the study.",{"count":159,"type":20},198,[23,24],"For patients with active proliferative IgG4-RD, we plan to conduct a 52-week prospective randomized controlled trial to compare the efficacy and safety of 5mg lenalidomide plus prednisone, 10mg lenalidomide plus prednisone, and prednisone alone, so as to find new treatment measures for patients with proliferative IgG4-RD.",[82],"2025-12-29",{"date":165,"type":32},"2025-12-30",{"date":167,"type":32},"2025-07-07",{"date":169,"type":20},"2028-08-20",{"name":171,"class":39},"Peking Union Medical College Hospital",{"id":173,"slug":174,"hasResults":11,"nctId":175,"briefTitle":176,"officialTitle":177,"acronym":4,"eligibilityCriteria":178,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":179,"enrollmentInfo":180,"targetDuration":4,"studyType":21,"phases":182,"briefSummary":184,"conditions":185,"keywords":4,"overallStatus":28,"whyStopped":4,"lastUpdateSubmitDate":186,"lastUpdatePostDateStruct":187,"startDateStruct":189,"completionDateStruct":190,"leadSponsor":192,"locationsCount":94},"100615898","early-phase-1-an-exploratory-clinical-study-on-the-safety-and-efficacy-of-cd19bcma-car-nk-in-the-treatment-of-relapsed-and-refractory-igg4-related-disease-100615898","NCT07298590","An Exploratory Clinical Study on the Safety and Efficacy of CD19\u002FBCMA CAR-NK in the Treatment of Relapsed and Refractory IgG4-related Disease","An Exploratory Clinical Study on the Safety and Efficacy of CD19\u002FBCMA CAR-NK (KN5601) in the Treatment of Relapsed and Refractory IgG4-related Disease (IgG4-RD)","Inclusion Criteria:\n\n1. Subjects must be able to understand and provide informed consent and be willing to comply with study procedures and follow-up.\n2. The age at the time of signing the informed consent must be at least 18 years old and no more than 70 years old.\n3. Meet the 2020 Japanese criteria or ACR\u002FEULAR IgG4-RD classification criteria.\n4. Subjects with relapsed\u002Frefractory active IgG4-RD at screening on an IgG4-RD RI ≥4, simultaneously meeting the following definitions of relapse or refractory disease:\n\n   1. Definitions of relapse: subjects with IgG4-RD achieved remission after treatment but was active again before screening, and were classified as a high-risk group for recurrence assessed by assessment committee ;\n   2. Definitions of before screening: subjects had used glucocorticoids or glucocorticoids combined with at least one conventional synthetic disease-modifying antirheumatic drug (csDMARDs) (including cyclophosphamide, mycophenolate mofetil, azathioprine, methotrexate, cyclosporine, tacrolimus, sirolimus, leflunomide, elorimod, thalidomide, etc.), or at least one approved biologic agent (bDMARDs) (including rituximab, abatacept, etanercept, belimumab, etc.), or targeted synthetic (ts) DMARDs (including tofacitinib, upadacitinib, baricitinib, abrocitinib, deucravacitinib, etc.) for treatment, with a total treatment duration of ≥3 months, yet still in an active disease state, ineffective, intolerant, or experiencing relapse during glucocorticoid tapering.\n5. No history of severe allergic reaction.\n6. Female participants of childbearing age must have a negative pregnancy test upon enrollment in the study; indeterminate results will not be accepted.\n7. Female subjects of childbearing age and male subjects with female partners of childbearing potential must agree to consistently use effective methods of birth control within 6 months after the last KN5601 infusion.\n8. Echocardiography show that the heart structure is basically normal and the left ventricular ejection fraction (LVEF) is ≥55%; no obvious abnormalities are found on the electrocardiogram.\n9. Pulmonary function: No severe lung disease, SpO2 ≥ 92%.\n10. All subjects' eligibility for enrollment must be confirmed by an independent Assessment Committee (AC) (the committee consists of independent data monitors and clinical experts separate from the study). They will review the eligibility of each patient based on the scores entered at screening, as well as brief descriptions provided by the investigators regarding the supporting diagnosis, scores, and the patient's clinical status in relation to enrollment criteria..\n\nExclusion Criteria:\n\n1. Presence of a condition other than IgG4-RD that (e.g., asthma) is likely to require systemic Glucocorticoids (GC) for disease control during the period of the trial.\n2. Malignancy within 5 years (except successfully treated in situ cancer, resected squamous cell or basal cell carcinoma of the skin.).\n3. During the screening visit, one of the following laboratory test values must be met, except for those caused by IgG4-RD.:\n\n   1. Serum aspartate aminotransferase (AST) or alanine aminotransferase (ALT) greater than three times the upper limit of normal (ULN).\n   2. Total bilirubin \\> two times the ULN unless caused by Gilbert's disease. Gilbert's disease with total bilirubin \\> three times ULN.\n   3. White blood cell (WBC) count \\\u003C3.0×10⁹\u002FL;\n   4. Absolute neutrophil count (ANC) \\\u003C1.5×10⁹\u002FL;\n   5. Hemoglobin \\\u003C90 g\u002FL;\n   6. Platelet count \\\u003C75×10⁹\u002FL;\n   7. Estimated glomerular filtration rate (eGFR) ≤ 45 ml\u002F(min·1.73m2).\n4. Evidence suggests the presence of another uncontrolled disease, which the investigator has determined may affect the subject's participation in the trial.\n5. Active infection requiring hospitalization or treatment with systemic antimicrobial agents within the 30 days prior to treatment allocation\u002Frandomization.\n6. Received rituximab or other B-cell depleting therapies within 6 months prior to the baseline visit, unless B cells have recovered (B-cell recovery is defined as peripheral blood B-cell count ≥ the lower limit of normal reference range or returned to pre-treatment levels)。\n7. The use of supplemental oxygen at baseline.\n8. During the screening visit or within 90 days prior to the screening visit: T-SPOT positive. If the result is indeterminate, the T-SPOT must be repeated (using the same or a different T-SPOT) and shown as negative.\n9. During screening visits, individuals with a history of chronic infection or serological evidence, including:\n\n   1. Human immunodeficiency virus infection;\n   2. Hepatitis B as indicated by surface antigen or hepatitis B core antibody positivity;\n   3. Hepatitis C as indicated by anti-hepatitis C antibody positivity; if a participant is Hepatitis C antibody positive, they will be eligible to participate in the study if he\u002Fshe is negative for viral load at screening.\n10. Planned vaccination with live vaccines during the trial.\n11. Participant is pregnant or breastfeeding, or planning a pregnancy while enrolled in the study.\n12. IgG4-RD that is dominated primarily by advanced fibrotic lesions. Specifically, participants whose disease manifestations consist only of\n\n    1. retroperitoneal fibrosis,\n    2. fibrosing mediatinitis,\n    3. sclerosing mesenteritis, and\n    4. Riedel's thyroiditis. Subjects were eligible to be included only if they had non-advanced fibrotic disease in at least one organ system and otherwise met the inclusion and exclusion criteria.\n13. Evidence a SARS-CoV-2 (COVID-19) infection started within the 30 days prior to treatment allocation\u002Frandomization. Participants diagnosed with SARS-CoV-2 (COVID-19) infection more than 30 days prior to treatment .allocation\u002Frandomization must have symptoms resolved and be deemed fit to participate in the trial.\n14. Researchers consider any situations that may increase the risk to participants or interfere with the trial results (including but not limited to a history of mental illness, alcoholism, drug abuse, poor compliance, etc.).","70 Years",{"count":181,"type":20},18,[183],"EARLY_PHASE1","A single arm, open-label pilot study is designed to determine the safety and effectiveness of CD19\u002FBCMA CAR NK cells (KN5601) in patients with IgG4 related diseases.",[82],"2025-12-21",{"date":188,"type":32},"2025-12-23",{"date":165,"type":20},{"date":191,"type":20},"2028-12-26",{"name":193,"class":39},"Changhai Hospital",{"id":195,"slug":196,"hasResults":11,"nctId":197,"briefTitle":198,"officialTitle":199,"acronym":200,"eligibilityCriteria":201,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":102,"enrollmentInfo":202,"targetDuration":4,"studyType":21,"phases":204,"briefSummary":205,"conditions":206,"keywords":208,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":212,"lastUpdatePostDateStruct":213,"startDateStruct":215,"completionDateStruct":217,"leadSponsor":219,"locationsCount":94},"100604382","phase-2-exploratory-study-of-anti-bcma-cd19-car-t-cell-therapy-in-relapsed-or-refractory-igg4-related-disease-100604382","NCT07148791","Exploratory Study of Anti-BCMA-CD19 CAR-T Cell Therapy in Relapsed or Refractory IgG4-Related Disease","Exploratory Clinical Study of Anti-BCMA-CD19 CAR-T Cell Therapy for Relapsed\u002FRefractory IgG4-Related Disease","BC19IGG4","Inclusion Criteria:\n\nTo participate, subjects must meet all of the following criteria:\n\n1. Aged 18 to 75 years, inclusive, regardless of sex.\n2. Meet the 2019 ACR\u002FEULAR classification criteria for IgG4-related disease.\n3. Involvement of two or more important systems\u002Fsites (including but not limited to the pancreas, bile ducts, kidneys and dura mater).\n4. Relapsed or refractory IgG4-RD: The disease either remains active after 3 months of glucocorticoid and\u002For rituximab therapy or relapses within 6 months post-treatment.\n5. Important organ function meeting the following conditions:\n\n   * Bone marrow: (i) neutrophil count ≥1×10\\^9\u002FL (excluding disease-related neutropenia); (ii) hemoglobin ≥60 g\u002FL.\n   * Hepatic function: ALT≤3×ULN (elevation caused by disease may be excluded); AST≤3×ULN (elevation caused by disease may be excluded); TBIL≤1.5×ULN (elevation caused by disease may be excluded).\n   * Renal function: creatinine clearance (Cockcroft-Gault formula) ≥30 ml\u002Fmin (excluding acute decline due to disease).\n   * Coagulation: international normalized ratio (INR) ≤ 1.5×ULN, prothrombin time (PT) ≤ 1.5×ULN\n   * Cardiac function: stable hemodynamics.\n6. Women of childbearing potential and male subjects with partners of childbearing potential must use medically accepted contraception or abstain during study treatment and for at least 12 months after the end of treatment. Women of childbearing potential must have a negative serum HCG test within 7 days before enrollment and must not be breastfeeding.\n7. Voluntary participation in this clinical study with signed informed consent and willingness to comply with study procedures and follow-up.\n8. Patent superficial peripheral veins adequate for intravenous infusion.\n\nExclusion Criteria:\n\nSubjects will be excluded if any of the following criteria are met:\n\n1. History of severe drug allergy or allergic constitution.\n2. Current or suspected uncontrollable or treatment-requiring fungal, bacterial, viral or other infections.\n3. Central nervous system disease (excluding disease-related epilepsy, psychosis, organic brain syndrome, cerebrovascular accident, encephalitis or central nervous system vasculitis).\n4. Cardiac insufficiency that precludes participation.\n5. Congenital immunoglobulin deficiency.\n6. Congenital malformation or nutritional disorder causing severe organ impairment.\n7. History of malignancy within the past five years.\n8. End-stage renal failure.\n9. Positive hepatitis B surface antigen and hepatitis B core antibody with HBV-DNA titers above the assay limit of detection; positive hepatitis C antibody with HCV-RNA positivity; positive human immunodeficiency virus antibody; positive syphilis serology.\n10. Psychiatric disorders or severe cognitive impairment.\n11. Participation in other clinical trials within three months before enrollment.\n12. Receipt of any investigational drug within 12 weeks before screening or within five half-lives of the agent (whichever is longer).\n13. Pregnant or intending to become pregnant.\n14. Any other reason deemed by the investigator to preclude enrollment.",{"count":203,"type":20},9,[23],"The goal of this clinical trial is to test the safety and potential benefit of a new immune cell therapy called anti-BCMA-CD19 CAR-T cells in adults (18-75 years) with IgG4-related disease (IgG4-RD) that has come back or not improved after standard treatments such as glucocorticoids or rituximab.\n\nThe main questions this study aims to answer are:\n\n* What medical problems (side effects) occur after receiving anti-BCMA-CD19 CAR-T cell therapy?\n* Does anti-BCMA-CD19 CAR-T cell therapy improve IgG4-RD disease activity scores at 12 weeks and 26 weeks?\n\nParticipants will:\n\n* Have their own blood immune cells collected by a procedure called leukapheresis\n* Receive short-term chemotherapy to prepare the immune system\n* Receive one intravenous infusion of anti-BCMA-CD19 CAR-T cells\n* Return for regular clinic visits over 26 weeks for safety checks, blood tests, and imaging\n* May be followed for up to one year in total",[82,207],"B-cell Mediated Autoimmune Disorders",[209,210,211],"IgG4 related disease","CAR-T therapy","BCMA\u002FCD19 CAR-T","2025-09-01",{"date":214,"type":32},"2025-09-03",{"date":216,"type":20},"2025-09-05",{"date":218,"type":20},"2029-12-31",{"name":220,"class":39},"Chinese PLA General Hospital",{"id":222,"slug":223,"hasResults":11,"nctId":224,"briefTitle":225,"officialTitle":226,"acronym":4,"eligibilityCriteria":227,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":228,"enrollmentInfo":229,"targetDuration":4,"studyType":21,"phases":231,"briefSummary":233,"conditions":234,"keywords":235,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":236,"lastUpdatePostDateStruct":237,"startDateStruct":239,"completionDateStruct":241,"leadSponsor":242,"locationsCount":94},"100567087","short-term-glucocorticoid-combined-with-mmf-for-igg4-rd-100567087","NCT06663618","Short-term Glucocorticoid Combined with MMF for IgG4-RD","Comparison of Short-term Glucocorticoid Monotherapy and Short-term Glucocorticoid Combined with Mycophenolate Mofetil in the Treatment of IgG4 Related Disease","Inclusion Criteria:\n\n1. 18-80 years old;\n2. All patients must meet the comprehensive diagnostic criteria of IgG4-RD revised in Japan in 2020 or the classification criteria of IgG4-RD formulated by ACR\u002FEULAR in 2019;\n3. Active IgG4-RD (at least one organ has an IgG4-RD reaction score \\>=2 at the time of enrollment.)；\n4. No previous medication or recurrence after withdrawal.\n\nExclusion Criteria:\n\n1. Combined with other autoimmune diseases as the main diagnosis.\n2. Pregnant or lactating women\n3. Patients with malignant tumor\n4. Active bacterial, fungal, viral or mycobacterial infections.\n5. Severe complications of important organs, and the expected survival time is less than 6 months.","80 Years",{"count":230,"type":20},63,[232],"NA","Comparison of short-term glucocorticoid monotherapy and short-term glucocorticoid combined with MMF in the treatment of IgG4 Related Disease",[55],[139],"2024-10-26",{"date":238,"type":32},"2024-10-29",{"date":240,"type":32},"2023-09-01",{"date":212,"type":20},{"name":243,"class":39},"Wen Zhang",{"id":245,"slug":246,"hasResults":11,"nctId":247,"briefTitle":248,"officialTitle":249,"acronym":4,"eligibilityCriteria":250,"healthyVolunteers":251,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":252,"targetDuration":4,"studyType":21,"phases":253,"briefSummary":254,"conditions":255,"keywords":4,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":257,"lastUpdatePostDateStruct":258,"startDateStruct":260,"completionDateStruct":262,"leadSponsor":264,"locationsCount":94},"100554305","early-phase-1-safety-and-efficacy-of-prg-2311-for-refractory-lupus-nephritis-and-igg4-related-disease-100554305","NCT06497361","Safety and Efficacy of PRG-2311 for Refractory Lupus Nephritis and IgG4-Related Disease","Safety and Efficacy of PRG-2311 (CD19\u002FBCMA-targeting CAR-T Cells) for Refractory Lupus Nephritis and IgG4-Related Disease","Inclusion Criteria:\n\n1. Age ≥ 18 years old;\n2. If the kidneys are involved, estimate the glomerular filtration rate (eGFR) to be ≥ 15 mL\u002Fminute\u002F1.73 m2;\n3. The following test values within 3 days before the collection of mononuclear cells meet the following standards:\n\n   1. Absolute lymphocyte count: ≥ 0.5 × 10 \\^ 9\u002FL \\[The use of granulocyte colony-stimulating factor (G CSF) is allowed, but subjects are not allowed to receive this supportive treatment within 7 days before the screening period laboratory examination\\];\n   2. Absolute neutrophil count: ≥ 1.0 × 10 \\^ 9\u002FL \\[The use of granulocyte colony-stimulating factor (G-CSF) is allowed, but subjects are not allowed to receive this supportive treatment within 7 days before the screening period laboratory examination\\];\n   3. Platelets: Subject platelet count ≥ 50 × 10 \\^ 9\u002FL (subjects are not allowed to receive blood transfusion support within 7 days before the screening period laboratory examination);\n   4. Hemoglobin: ≥ 8.0 g\u002FdL (allowing the use of recombinant human erythropoietin) \\[subjects have not received red blood cell (RBC) infusion within 7 days prior to the screening period laboratory examination\\];\n   5. Creatinine clearance rate: (CrCl) or glomerular filtration rate (GFR) (Cockcroft Gault formula) ≥ 30 mL\u002Fmin;\n   6. Total bilirubin (serum): Total bilirubin (serum) ≤ 1.5 × ULN; Blood bilirubin\\>1.5 × Gilbert subjects from ULN can be enrolled with the consent of the sponsor AST and ALT: ≤ 3.0 × ULN;\n   7. Plasma prothrombin time (PT), international standardized ratio (INR), partial prothrombin time (APTT): PT ≤ 1.5 × ULN, APTT ≤ 1.5 × ULN, INR ≤ 1.5 × ULN Willing to sign an informed consent form.\n4. Fertile men and women of childbearing age must agree to use effective contraception from the time they sign an informed consent and up to 1 year after the study drug is used. Blood pregnancy tests for women of reproductive age at the time of screening and before cell infusion must be negative.\n5. The patients or their guardians agree to participate in the clinical study and sign the informed consent, indicating that they understand the purpose and procedure of the clinical study and are willing to participate in the study.\n\n   * for refractory LN\n\n     1. According to the 2019 American Society of Rheumatology (ACR) criteria, diagnosed with systemic lupus erythematosus, within 6 months prior to infusion, confirmed by renal tissue biopsy according to the 2003 International Society of Nephrology (ISN)\u002FSociety of Nephropathology (RPS) criteria as active, proliferative lupus nephritis (LN), type III or IV, or type III\u002FIV combined with type V, or type V. And have received standard treatment that is ineffective or relapses after disease remission.\n     2. Positive anti-nuclear antibodies (ANA) and\u002For anti-dsDNA antibodies during the screening period.\n     3. The SLE Disease Activity Index (SLEDAI-2000) score during the screening period is ≥ 8. SLEDAI-2000 clinical score ≥ 6 points, but low complement and\u002For anti ds-DNA positivity can be selected.\n   * for refractory IgG4-RD\n\n     1. According to the 2019 ACR\u002FEULAR criteria, diagnosed with IgG4-RD;\n     2. The clinical manifestations were recurrent or refractory IgG4-RD;\n     3. IgG4-RD response index (RI) ≥2, the disease is in the active stage;\n     4. meet the clinical phenotype of Mikulitz\u002Fsystemic\n\nExclusion Criteria:\n\nSubjects who meet any of the following criteria should be excluded from this study:\n\n1. Pregnant or lactating women;\n2. A history of malignant tumors within 5 years （①Carcinoma in situ of the cervix that has undergone curative treatment for more than 12 months prior to screening, ②Basal cell or squamous cell carcinoma of the skin that has been treated therapeutically, ③ Prostate cancer that has been treated with radical prostatectomy or curative radiation therapy for more than 3 years prior to screening has no known recurrence and is not currently receiving treatment；④have had surgery for thyroid cancer, and have not evidence of active disease）;\n3. Received any B-cell depletion biologic therapy (for example, rituximab, ocrelizumab, obinutuzumab, ofatumumab, inebilizumab, etc) in the 6 months prior to CAR-T reinfusion, unless B-cell recovery was demonstrated;\n4. Received immunosuppressant therapy within 3 days prior to CAR-T reinfusion, or systemic corticosteroid therapy (\\>10 mg\u002F day of prednisone or equivalent doses of other corticosteroids) within 3 days prior to CAR-T reinfusion;\n5. Received live vaccine or live therapeutic STDS within 2 weeks prior to screening;\n6. The presence of chronic and active hepatitis B (except for HBV DNA testing below 500IU\u002Fml), hepatitis C (HCV), human immunodeficiency virus (HIV) infection, or syphilis infection;\n7. With an active infection that requires intravenous antibiotics or hospitalization;\n8. Obvious evidence of cardiovascular disease as follows: a N-terminal B-type natriuretic peptide (NT proBNP)\\>8500ng\u002FL; b. The New York Heart Association (NYHA) classifies heart failure as Grade IV; c. Patients who received hospitalization for unstable angina or myocardial infarction within 6 months prior to the first administration, or patients who received percutaneous cardiac intervention and received the most recent stent placement within 6 months or coronary artery bypass grafting within 6 months;\n9. People who have a known allergy, hypersensitivity, intolerance, or contraindication to any component of PRG-2311 or the drugs that may be used in the study, including fludarabine, cyclophosphamide, tolumab, or albumin, or who have had a prior severe allergic reaction;\n10. Patients with other conditions determined by the investigator to be unsuitable for lymphocyte clearance or cell infusion, or who are otherwise unsuitable for study participation.",true,{"count":104,"type":20},[183],"A Clinical Study on the Safety and Effectiveness of CD19\u002FBCMA Chimeric Antigen Receptor T Cells in the Treatment of Refractory Lupus Nephritis and IgG4-Related Disease.",[256,55],"Lupus Nephritis","2024-07-04",{"date":259,"type":32},"2024-07-11",{"date":261,"type":20},"2024-07",{"date":263,"type":20},"2028-07",{"name":265,"class":39},"Tongji Hospital",{"id":267,"slug":268,"hasResults":11,"nctId":269,"briefTitle":270,"officialTitle":271,"acronym":4,"eligibilityCriteria":272,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":273,"targetDuration":4,"studyType":21,"phases":274,"briefSummary":275,"conditions":276,"keywords":4,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":257,"lastUpdatePostDateStruct":277,"startDateStruct":278,"completionDateStruct":279,"leadSponsor":280,"locationsCount":94},"100554307","early-phase-1-safety-and-efficacy-of-prg-1801-for-refractory-lupus-nephritis-and-igg4-related-disease-100554307","NCT06497387","Safety and Efficacy of PRG-1801 for Refractory Lupus Nephritis and IgG4-Related Disease","Safety and Efficacy of PRG-1801(BCMA-targeting CAR-T Cells) for Refractory Lupus Nephritis and IgG4-Related Disease","Inclusion Criteria:\n\nA. Age ≥ 18 years old; B. If the kidneys are involved, estimate the glomerular filtration rate (eGFR) to be ≥ 15 mL\u002Fminute\u002F1.73 m2;\n\nC. The following test values within 3 days before the collection of mononuclear cells meet the following standards:\n\n1. Absolute lymphocyte count: ≥ 0.5 × 10 \\^ 9\u002FL \\[The use of granulocyte colony-stimulating factor (G CSF) is allowed, but subjects are not allowed to receive this supportive treatment within 7 days before the screening period laboratory examination\\];\n2. Absolute neutrophil count: ≥ 1.0 × 10 \\^ 9\u002FL \\[The use of granulocyte colony-stimulating factor (G-CSF) is allowed, but subjects are not allowed to receive this supportive treatment within 7 days before the screening period laboratory examination\\];\n3. Platelets: Subject platelet count ≥ 50 × 10 \\^ 9\u002FL (subjects are not allowed to receive blood transfusion support within 7 days before the screening period laboratory examination);\n4. Hemoglobin: ≥ 8.0 g\u002FdL (allowing the use of recombinant human erythropoietin) \\[subjects have not received red blood cell (RBC) infusion within 7 days prior to the screening period laboratory examination\\];\n5. Creatinine clearance rate: (CrCl) or glomerular filtration rate (GFR) (Cockcroft Gault formula) ≥ 30 mL\u002Fmin;\n6. Total bilirubin (serum): Total bilirubin (serum) ≤ 1.5 × ULN; Blood bilirubin\\>1.5 × Gilbert subjects from ULN can be enrolled with the consent of the sponsor AST and ALT: ≤ 3.0 × ULN;\n7. Plasma prothrombin time (PT), international standardized ratio (INR), partial prothrombin time (APTT): PT ≤ 1.5 × ULN, APTT ≤ 1.5 × ULN, INR ≤ 1.5 × ULN Willing to sign an informed consent form.\n8. Fertile men and women of childbearing age must agree to use effective contraception from the time they sign an informed consent and up to 1 year after the study drug is used. Blood pregnancy tests for women of reproductive age at the time of screening and before cell infusion must be negative.\n9. The patients or their guardians agree to participate in the clinical study and sign the informed consent, indicating that they understand the purpose and procedure of the clinical study and are willing to participate in the study.\n\n   * for refractory LN\n\nA. According to the 2019 American Society of Rheumatology (ACR) criteria, diagnosed with systemic lupus erythematosus, within 6 months prior to infusion, confirmed by renal tissue biopsy according to the 2003 International Society of Nephrology (ISN)\u002FSociety of Nephropathology (RPS) criteria as active, proliferative lupus nephritis (LN), type III or IV, or type III\u002FIV combined with type V, or type V. And have received standard treatment that is ineffective or relapses after disease remission.\n\nB. Positive anti-nuclear antibodies (ANA) and\u002For anti-dsDNA antibodies during the screening period.\n\nC. The SLE Disease Activity Index (SLEDAI-2000) score during the screening period is ≥ 8. SLEDAI-2000 clinical score ≥ 6 points, but low complement and\u002For anti ds-DNA positivity can be selected.\n\n-for refractory IgG4-RD\n\nA. According to the 2019 ACR\u002FEULAR criteria, diagnosed with IgG4-RD; B. The clinical manifestations were recurrent or refractory IgG4-RD; C. IgG4-RD response index (RI) ≥2, the disease is in the active stage; D. meet the clinical phenotype of Mikulitz\u002Fsystemic\n\nExclusion Criteria:\n\nSubjects who meet any of the following criteria should be excluded from this study:\n\n1. Pregnant or lactating women;\n2. A history of malignant tumors within 5 years (① subjects with cervical carcinoma in situ who have been completely removed and have not experienced recurrence or metastasis for at least 3 years may participate in this study. ② subjects with basal cell or squamous cell carcinoma who have been completely removed and have not experienced recurrence for at least 3 years may participate in this study);（①Carcinoma in situ of the cervix that has undergone curative treatment for more than 12 months prior to screening, ②Basal cell or squamous cell carcinoma of the skin that has been treated therapeutically, ③ Prostate cancer that has been treated with radical prostatectomy or curative radiation therapy for more than 3 years prior to screening has no known recurrence and is not currently receiving treatment；④have had surgery for thyroid cancer, and have not evidence of active disease）;\n3. Received any B-cell depletion biologic therapy (for example, rituximab, ocrelizumab, obinutuzumab, ofatumumab, inebilizumab, etc) in the 6 months prior to CAR-T reinfusion, unless B-cell recovery was demonstrated;\n4. Received immunosuppressant therapy within 3 days prior to CAR-T reinfusion, or systemic corticosteroid therapy (\\>10 mg\u002F day of prednisone or equivalent doses of other corticosteroids) within 3 days prior to CAR-T reinfusion;\n5. Received live vaccine or live therapeutic STDS within 2 weeks prior to screening;\n6. The presence of chronic and active hepatitis B (except for HBV DNA testing below 500IU\u002Fml), hepatitis C (HCV), human immunodeficiency virus (HIV) infection, or syphilis infection;\n7. With an active infection that requires intravenous antibiotics or hospitalization;\n8. Obvious evidence of cardiovascular disease as follows: a N-terminal B-type natriuretic peptide (NT proBNP)\\>8500ng\u002FL; b. The New York Heart Association (NYHA) classifies heart failure as Grade IV; c. Patients who received hospitalization for unstable angina or myocardial infarction within 6 months prior to the first administration, or patients who received percutaneous cardiac intervention and received the most recent stent placement within 6 months or coronary artery bypass grafting within 6 months;\n9. People who have a known allergy, hypersensitivity, intolerance, or contraindication to any component of PRG-1801 or the drugs that may be used in the study, including fludarabine, cyclophosphamide, tolumab, or albumin, or who have had a prior severe allergic reaction;\n10. Patients with other conditions determined by the investigator to be unsuitable for lymphocyte clearance or cell infusion, or who are otherwise unsuitable for study participation.",{"count":104,"type":20},[183],"A Clinical Study on the Safety and Effectiveness of BCMA Chimeric Antigen Receptor T Cells in the Treatment of Refractory Lupus Nephritis and IgG4-Related Disease.",[256,55],{"date":259,"type":32},{"date":261,"type":20},{"date":263,"type":20},{"name":265,"class":39},{"id":282,"slug":283,"hasResults":11,"nctId":284,"briefTitle":285,"officialTitle":286,"acronym":4,"eligibilityCriteria":287,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":228,"enrollmentInfo":288,"targetDuration":4,"studyType":21,"phases":290,"briefSummary":291,"conditions":292,"keywords":293,"overallStatus":28,"whyStopped":4,"lastUpdateSubmitDate":296,"lastUpdatePostDateStruct":297,"startDateStruct":299,"completionDateStruct":301,"leadSponsor":303,"locationsCount":4},"100496627","study-of-sirolimus-in-igg4-related-disease-100496627","NCT05746689","Study of Sirolimus in IgG4-related Disease","Combination Therapy of Sirolimus and Glucocorticoids for the Maintenance of Remission in Patients With IgG4-related Disease","Inclusion criteria:\n\n1. Patients diagnosed with IgG4-RD according to the 2011 Comprehensive Diagnostic Criteria for IgG4-RD;\n2. Status classified as active disease based on an IgG4-RD Responder Index (RI) ≥2 at screening.\n\n1.Exclusion criteria: 2.Having used glucocorticoids (equivalent to more than 10mg per day of prednisone), immunosuppressant or biologic within 3 months prior to enrollment; 3.Having any contraindication of glucocorticoids or sirolimus, or allergy to sirolimus, or having experienced serious adverse reactions from previous use of any of the above drugs; 4.Combined with other connective disease; 5.History or evidence of a clinically unstable\u002Funcontrolled disorder, condition or disease (including but not limited to cardiopulmonary, oncologic, renal, hepatic, metabolic, hematologic or psychiatric) other than IgG4-RD that, in the opinion of the Investigator, would pose a risk to patient safety or interfere with the study evaluation, procedures or completion; 6.Active infection, including hepatitis B virus, hepatitis C virus, and tuberculosis; 7.Malignancy within 5 years; 8.Other serious complications or general conditions do not permit; 9.Pregnancy or to be pregnant, or breast feeding; 10.Unable to adhere to follow-up or the patient refuses to provide consent.",{"count":289,"type":20},20,[232],"gG4-related disease (IgG4-RD) is a newly recognized systemic autoimmune disease that can involve the pan-creatobiliary tract, retroperitoneum\u002Faorta, head and neck region, and salivary glands, et al. Glucocorticoids are the first-line agents for the treatment of IgG4-RD, however, in order to maintain long-term disease stability and avoid disease relapse, glucocorticoids maintenance therapy should last for a long period, which may induce various glucocorticoid-associated adverse reactions. Sirolimus plays dual roles in inhibiting lymphocyte activation and fibroblast proliferation. It is inferred from its mechanism that sirolimus is a good potential treatment option for IgG4-RD. Therefore, we conducted this single-arm clinical trial on patients with IgG4-RD to determine the efficacy and safety of sirolimus.",[55],[139,294,295],"sirolimus","mTOR","2023-02-25",{"date":298,"type":32},"2023-02-28",{"date":300,"type":20},"2023-03-01",{"date":302,"type":20},"2028-12-31",{"name":304,"class":39},"Peking University International Hospital",{"id":306,"slug":307,"hasResults":11,"nctId":308,"briefTitle":309,"officialTitle":310,"acronym":4,"eligibilityCriteria":311,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":312,"enrollmentInfo":313,"targetDuration":315,"studyType":316,"phases":4,"briefSummary":317,"conditions":318,"keywords":4,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":323,"lastUpdatePostDateStruct":324,"startDateStruct":326,"completionDateStruct":328,"leadSponsor":330,"locationsCount":94},"100322131","meir-medical-center-rheumatologic-biobank-100322131","NCT03473912","Meir Medical Center Rheumatologic Biobank","Rheumatologic Biobank for Clinical and Basic Research: Isolation, Extraction and Storage of Protein, DNA and RNA Extracted From Tissues of Patients With Rheumatoid Diseases","Inclusion Criteria:\n\n* above 18 years of age\n* willing to sign informed consent\n* have a rheumatoid condition\n\nExclusion Criteria:\n\n\\-","120 Years",{"count":314,"type":20},500,"20 Years","OBSERVATIONAL","Serum, synovial fluid and skin biopsies from patients will be collected to the biobank with rheumatoid diseases. These samples will later be used for clinical and basic research, following approval of each specific study by the IRB. The investigators intend to extract protein, DNA and RNA from each sample.",[319,320,321,55,322],"Rheumatoid Arthritis","Lupus Erythematosus, Systemic","Systemic Sclerosis","Sjogren's Syndrome","2019-03-07",{"date":325,"type":32},"2019-03-11",{"date":327,"type":32},"2018-04-16",{"date":329,"type":20},"2037-11",{"name":331,"class":39},"Meir Medical Center",{"id":333,"slug":334,"hasResults":11,"nctId":335,"briefTitle":336,"officialTitle":337,"acronym":4,"eligibilityCriteria":338,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":102,"enrollmentInfo":339,"targetDuration":4,"studyType":316,"phases":4,"briefSummary":341,"conditions":342,"keywords":343,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":344,"lastUpdatePostDateStruct":345,"startDateStruct":347,"completionDateStruct":349,"leadSponsor":351,"locationsCount":94},"100183999","a-prospective-cohort-study-of-igg4rd-in-china-100183999","NCT01670695","A Prospective Cohort Study of IgG4RD in China","Cohort Study of Patients With IgG4-Related Disease","Inclusion Criteria:\n\n* Males and females\n* Age 18-75 years old with informed consent\n* Patients with IgG4-RD:\n\n  1. swelling, sclerosing and inflammatory involvement of one or more organ, including sclerosing pancreatitis, sclerosing cholangitis, inflammatory pseudotumors, retroperitoneal or mediastinal fibrosis, interstitial nephritis, hypophysitis, sclerosing dacryoadenitis, sialadenitis, inflammatory aortic aneurysm, lymphadenopathy, or other inflammatory conditions;\n  2. elevated serum IgG4 (\\>1.35 g\u002FL)\n  3. histopathologic features of fibrosis and\u002For lymphocytic and polyclonal plasma cell infiltration (and IgG4+ plasma cells on immunohistology when performed);\n  4. exclusion of other diseases.\n\nExclusion Criteria:\n\n* Females planning to bear a child recently or with childbearing potential\n* Concurrent severe and\u002For uncontrolled and\u002For unstable diseases\n* Patient with malignancy",{"count":340,"type":20},1000,"This is an cohort study to investigate the disease course and treatment response of patients with IgG4-related disease.",[55],[139,111],"2017-04-07",{"date":346,"type":32},"2017-04-11",{"date":348,"type":32},"2012-01",{"date":350,"type":20},"2032-01",{"name":171,"class":39},{"id":353,"slug":354,"hasResults":11,"nctId":355,"briefTitle":356,"officialTitle":356,"acronym":4,"eligibilityCriteria":357,"healthyVolunteers":11,"sex":16,"minAge":4,"maxAge":4,"enrollmentInfo":358,"targetDuration":360,"studyType":316,"phases":4,"briefSummary":361,"conditions":362,"keywords":363,"overallStatus":58,"whyStopped":4,"lastUpdateSubmitDate":365,"lastUpdatePostDateStruct":366,"startDateStruct":368,"completionDateStruct":370,"leadSponsor":372,"locationsCount":94},"100287569","national-registry-of-igg4-rd-in-china-100287569","NCT03023371","National Registry of IgG4-RD in China","Inclusion Criteria:\n\n* Conforming to the diagnostic criteria of IgG4-RD (2011);\n\nExclusion Criteria:\n\n* Excluding other mimicing IgG4-RD, including tumors, vasculitis and sarcoidosis.",{"count":359,"type":20},900,"10 Years","The aim of this study is to establish a nation-wide cohort study of IgG4-related disease (IgG4-RD) in China.\n\nMethods: All the patients fulfilling diagnostic criteria of IgG4-RD (2011) would be enrolled from multi-centers around China. A online database system has been established.\n\nEndpoints: The primary endpoint is to investigate the clinical manifestations of Chinese IgG4-RD patients; the secondary endpoints including the demographic features,laboratory characteristics, immunological tests, imaging and pathological features, in addition, the treatment and prognosis of the disease.",[27],[364],"Registry; cohort study; IgG4-related disease","2017-01-23",{"date":367,"type":20},"2017-01-24",{"date":369,"type":4},"2016-12",{"date":371,"type":20},"2026-12",{"name":171,"class":39}]