[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"igg4-related-diseases\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:igg4-related-diseases":26},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,8,0,[8,52,84,107,137,161,206,237],{"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":4,"briefSummary":23,"conditions":24,"keywords":29,"overallStatus":39,"whyStopped":4,"lastUpdateSubmitDate":40,"lastUpdatePostDateStruct":41,"startDateStruct":44,"completionDateStruct":46,"leadSponsor":48,"locationsCount":51},"100634219","validation-of-the-odesa-criteria-2026-for-autoimmune-pancreatitis-100634219",false,"NCT07536841","Validation of the Odesa Criteria 2026 for Autoimmune Pancreatitis","Validation of the Odesa Criteria 2026 (OC26) for the Diagnosis of Autoimmune Pancreatitis","ОС26","Inclusion Criteria:\n\n* Adults (≥18 years) undergoing evaluation for suspected autoimmune pancreatitis.\n* Availability of clinical, serological, imaging, and\u002For histological data required for OC26 scoring.\n* Ability to provide informed consent (for prospective participants).\n\nExclusion Criteria:\n\n* Confirmed alternative diagnosis explaining pancreatic findings (e.g., pancreatic cancer, acute pancreatitis of other etiology).\n* Incomplete diagnostic data preventing OC26 scoring.\n* Prior pancreatic surgery altering diagnostic interpretation.","ALL","18 Years",{"count":20,"type":21},82,"ESTIMATED","OBSERVATIONAL","This ambispective observational study aims to validate the Odesa Criteria 2026 (OC26), a flexible domain-based point scoring system for diagnosing autoimmune pancreatitis. The study evaluates diagnostic accuracy and reproducibility of OC26 across clinical, serological, morphological, and histological domains.",[25,26,27,28],"Autoimmune Pancreatitis","IgG4-Related Diseases","Pancreatic Cancer","Chronic Pancreatitis",[30,31,32,33,34,35,36,37,38],"AIP","IgG4","Autoimmune pancreatitis diagnosis","Pancreatic imaging","Endoscopic ultrasound","Serum protein fractions","IgG6\u002FIgG8 ratio","Odesa Criteria 2026 (OC26)","EUS Doppler","RECRUITING","2026-05-23",{"date":42,"type":43},"2026-05-28","ACTUAL",{"date":45,"type":43},"2026-05-01",{"date":47,"type":21},"2026-12-01",{"name":49,"class":50},"Military Medical Clinical Center of the Southern Region, Ukraine","OTHER",2,{"id":53,"slug":54,"hasResults":11,"nctId":55,"briefTitle":56,"officialTitle":57,"acronym":58,"eligibilityCriteria":59,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":60,"enrollmentInfo":61,"targetDuration":4,"studyType":63,"phases":64,"briefSummary":66,"conditions":67,"keywords":4,"overallStatus":73,"whyStopped":4,"lastUpdateSubmitDate":74,"lastUpdatePostDateStruct":75,"startDateStruct":77,"completionDateStruct":79,"leadSponsor":81,"locationsCount":83},"100637500","a-study-to-evaluate-mtm-h-001-injection-in-adult-patients-with-b-cell-related-autoimmune-diseases-100637500","NCT07593911","A Study to Evaluate MTM-H-001 Injection in Adult Patients With B-Cell-Related Autoimmune Diseases","An Open-Label, Single-Arm, Dose-Escalation and Expansion Basket Study Evaluating the Safety, Tolerability, Pharmacokinetics (PK), Pharmacodynamics (PD), and Preliminary Efficacy of MTM-H-001 in Patients With B-Cell-Related Autoimmune Diseases (ArchAid-002)","ArchAid-002","Inclusion Criteria:\n\n* Key inclusion criteria include：\n\n  1. Male or female, aged 18 - 75 years old (inclusive);\n  2. The participant or his\u002Fher legally acceptable representative gives consent to this clinical study participation and signs an Informed Consent Form (ICF) indicating their understanding of the objectives and procedures of the clinical study and willingness to participate in the study;\n  3. Confirmed diagnosis of the target autoimmune disease per international classification criteria;\n  4. Adequate organ function to tolerate treatment;\n  5. Women of childbearing potential (WOCBP) must have a negative serum pregnancy test at screening and agree to use at least one effective contraceptive method throughout the study and within 6 months after the last treatment cycle.\n\nExclusion Criteria:\n\n* Key exclusion criteria include:\n\n  1. Active, severe, uncontrolled infection;\n  2. Pregnancy or breastfeeding women;\n  3. History of malignancy within the protocol-defined period, except adequately treated low-risk cancers;\n  4. Any condition that, in the investigator's opinion, would compromise safety, compliance, or interpretability of study data.","75 Years",{"count":62,"type":21},75,"INTERVENTIONAL",[65],"NA","This is an investigator-initiated, open-label, single-arm, dose-escalation and dose-expansion study to evaluate the safety, tolerability, PK, PD, and preliminary efficacy of MTM-H-001 in adult participants with B-cell-related autoimmune diseases.",[68,69,70,71,26,72],"Systemic Lupus Erthematosus (SLE)","Lupus Nephritis (LN)","Multiple Sclerosis","Myasthenia Gravis (MG)","Rheumatoid Arthritis (RA)","NOT_YET_RECRUITING","2026-05-12",{"date":76,"type":43},"2026-05-18",{"date":78,"type":21},"2026-06",{"date":80,"type":21},"2031-06",{"name":82,"class":50},"Beijing GoBroad Hospital",1,{"id":85,"slug":86,"hasResults":11,"nctId":87,"briefTitle":88,"officialTitle":89,"acronym":4,"eligibilityCriteria":90,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":60,"enrollmentInfo":91,"targetDuration":4,"studyType":63,"phases":93,"briefSummary":94,"conditions":95,"keywords":96,"overallStatus":39,"whyStopped":4,"lastUpdateSubmitDate":98,"lastUpdatePostDateStruct":99,"startDateStruct":101,"completionDateStruct":103,"leadSponsor":105,"locationsCount":83},"100600943","a-study-on-the-effect-of-mrd-detected-by-fapi-pet--ct-on-the-recurrence-of-igg4-rd-100600943","NCT07104058","A Study on the Effect of MRD Detected by FAPI PET \u002F CT on the Recurrence of IgG4-RD","A Prospective Study on the Effect of Minimal Residual Disease Detected by Fibroblast Activation Protein PET \u002F CT on the Recurrence of IgG4-related Disease","Inclusion Criteria:\n\n1. age 18-75 years old ;\n2. Meet the 2019 ACR \u002F EURLAR IgG4-RD diagnostic criteria ;\n3. The disease was stable for more than 1 year, and the clinical evaluation of the disease was complete remission \\* ;\n4. Glucocorticoid dosage ≤ prednisone 7.5mg \u002F d × 6 months or more, or combined with an immunosuppressive therapy ( mycophenolate mofetil ) Ester ≤ 1.0g \u002F d, leflunomide ≤ 20mg \u002F d, methotrexate ≤ 15mg \u002F w, azathioprine ≤ 100mg \u002F d, iguratimod ≤ 25mg Bid )\n\nExclusion Criteria:\n\n( 1 ) Combined with other connective tissue disease patients ( 2 ) patients with tumor ; ( 3 ) women during pregnancy or planning pregnancy ; ( 4 ) active infection, including HIV, HCV, HBV, TB, etc. ; ( 5 ) Severe irreversible organ damage ; ( 6 ) In the active stage of the disease, the IgG4-RD reaction index score of the single affected organ was 2 points ; ( 7 ) stable condition less than 1 year ; ( 8 ) patients who relapsed within 1 year of previous hormone reduction and withdrawal ; ( 9 ) Combination of two or more immunosuppressive agents.",{"count":92,"type":21},46,[65],"This study intends to use the new technology of fibroblast activation protein PET \u002F CT to study whether the tiny residual lesions detected by 68Ga-FAPI PET \u002F CT are related to the recurrence of IgG4-RD in patients with IgG4-RD who have been clinically evaluated for complete remission after treatment.",[26],[26,97],"FAPI PET\u002FCT","2025-08-03",{"date":100,"type":43},"2025-08-05",{"date":102,"type":43},"2025-05-10",{"date":104,"type":21},"2027-12-01",{"name":106,"class":50},"Luo Yaping",{"id":108,"slug":109,"hasResults":11,"nctId":110,"briefTitle":111,"officialTitle":112,"acronym":4,"eligibilityCriteria":113,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":114,"enrollmentInfo":115,"targetDuration":4,"studyType":63,"phases":117,"briefSummary":119,"conditions":120,"keywords":4,"overallStatus":39,"whyStopped":4,"lastUpdateSubmitDate":127,"lastUpdatePostDateStruct":128,"startDateStruct":130,"completionDateStruct":132,"leadSponsor":134,"locationsCount":83},"100591301","phase-1-a-clinical-study-of-yts109-cell-in-rr-autoimmune-diseases-100591301","NCT06978647","A Clinical Study of YTS109 Cell in R\u002FR Autoimmune Diseases","An Exploratory Clinical Study of YTS109 Cell in Subjects With Relapsed\u002FRefractory Autoimmune Diseases","Inclusion Criteria:\n\n* Subjects must meet both the following common inclusion criteria and disease-specific inclusion criteria simultaneously to be eligible for participation in this study:\n\nCommon inclusion criteria:\n\n1. Age ranges from 18 to 65 years old (including threshold), regardless of gender.\n2. Detection of positive CD19 expression on peripheral blood B cells by flow cytometry.\n3. Essential Organ Function Criteria:\n\n\u003C!-- -->\n\n1. Bone marrow: Neutrophils ≥1×10\\^9\u002FL (within 2 weeks, excluding granulocyte colony-stimulating factor use).\n\n   Hemoglobin ≥60 g\u002FL.\n2. Liver: ALT\u002FAST ≤3×ULN (disease-related elevations permitted). TBIL ≤1.5×ULN (disease-related elevations permitted).\n3. Renal: CrCl≥30mL\u002Fmin (Cockcroft-Gault formula, excluding acute declines).\n4. Coagulation: INR\u002FPT ≤1.5×ULN.\n5. Cardiovascular: Hemodynamic stability. 4. Fertile females or males with partners of childbearing age must use medically approved contraception or abstain during and ≥12 months post- treatment. Negative serum HCG test (within 7 days pre-enrollment) for fertile females; non-lactating.\n\n5\\. Voluntary participation with signed informed consent and compliance.\n\nSpecific inclusion criteria:\n\nRelapsing and refractory systemic lupus erythematosus:\n\n1\\. Meeting the EULAR\u002FACR 2019 SLE Classification Criteria; 2. SELENA SLEDAI≥6, or the presence of significant organ involvement, such as lupus nephritis (LN), etc; 3. Definition of relapsing and refractory condition: Persistence of disease activity or recurrence of disease activity after remission, despite undergoing treatment with a regimen containing at least two immunosuppressive agents (including glucocorticoids, cyclophosphamide, tacrolimus, mycophenolate mofetil (MMF), and cyclosporine) and\u002For biological agents for a minimum duration of two months.\n\nRelapsing and refractory Sjögren's syndrome:\n\n1. Meeting the 2002 American-European Consensus Group (AECG) criteria or the 2016 American College of Rheumatology\u002FEuropean League Against Rheumatism (ACR\u002FEULAR) classification criteria for primary Sjögren's syndrome;\n2. Having a disease activity score of EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) ≥ 6;\n3. Testing positive for anti-SSA\u002FRo antibodies;\n4. Definition of relapsing and refractory condition: Persistence of disease activity or recurrence of disease activity after remission, despite undergoing conventional treatment for more than six months. Definition of conventional treatment: Administration of glucocorticoids in combination with any of the following immunosuppressive agents or biological agents: cyclophosphamide, azathioprine, mycophenolate mofetil, methotrexate, leflunomide, tacrolimus, cyclosporine, as well as biological agents including rituximab, belimumab, telitacicept, etc.\n\nRelapsing and refractory Sjogren's Syndrome:\n\n1. Meeting the 2013 American College of Rheumatology (ACR) classification criteria for systemic sclerosis;\n2. Testing positive for systemic sclerosis-related antibodies;\n3. Presenting with diffuse cutaneous sclerosis or active interstitial lung disease (as indicated by ground-glass opacities on high-resolution computed tomography, HRCT);\n4. Definition of relapsing and refractory condition: Persistence of disease activity or recurrence of disease activity after remission, despite undergoing conventional treatment for more than six months. Definition of conventional treatment: Administration of glucocorticoids and cyclophosphamide, in combination with any one or more of the following immunomodulatory agents: antimalarial drugs, azathioprine, mycophenolate mofetil, methotrexate, leflunomide, tacrolimus, cyclosporine, as well as biological agents including rituximab, belimumab, telitacicept, etc.;\n5. Definition of progressive condition: Demonstrating rapid skin progression (an increase in modified Rodnan skin score, mRSS, of \\>25%) or pulmonary disease progression (a 10% decrease in forced vital capacity, FVC, or a \\>5% decrease in FVC accompanied by a 15% decrease in diffusion capacity for carbon monoxide, DLCO).\n\nNote: Meeting either criterion 4 or 5 is sufficient.\n\nRelapsing and refractory Inflammatory Myopathy:\n\n1. Meeting the 2017 European League Against Rheumatism\u002FAmerican College of Rheumatology (EULAR\u002FACR) classification criteria for inflammatory myopathies (including dermatomyositis, DM; polymyositis, PM; antisynthetase syndrome, ASS; and necrotizing myopathy, NM);\n2. Testing positive for myositis-specific antibodies;\n3. For patients with muscle involvement, having a Manual Muscle Testing-8 (MMT-8) score below 142 and at least two abnormal findings among the following five core measures (Physician's Global Assessment, PhGA; Patient's Global Assessment, PtGA, or extra-muscular disease activity score ≥ 2 points; Health Assessment Questionnaire, HAQ, total score ≥ 0.25; muscle enzyme levels 1.5 times the upper limit of normal range); or having an MMT-8 score ≥ 142 but presenting with active interstitial lung disease (as indicated by ground-glass opacities on high-resolution computed tomography, HRCT);\n4. Definition of relapsing and refractory condition: Persistence of disease activity or recurrence of disease activity after remission, despite undergoing conventional treatment for more than six months. Definition of conventional treatment: Administration of glucocorticoids and cyclophosphamide, in combination with any one or more of the following immunomodulatory agents: antimalarial drugs, azathioprine, mycophenolate mofetil, methotrexate, leflunomide, tacrolimus, cyclosporine, as well as biological agents including rituximab, belimumab, telitacicept, etc.;\n5. Definition of progressive condition: Demonstrating worsening myositis or rapidly progressive interstitial pneumonia.\n\nNote: Meeting either criterion 4 or 5 is sufficient.\n\nRelapsing and refractory Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis:\n\n1. Meeting the 2022 American College of Rheumatology\u002FEuropean League Against Rheumatism (ACR\u002FEULAR) diagnostic criteria for ANCA-associated vasculitis, including microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis.\n2. Testing positive for ANCA-related antibodies (either MPO-ANCA or PR3-ANCA positive).\n3. A Birmingham Vasculitis Activity Score (BVAS) of ≥15 points (out of a total of 63 points), indicating active vasculitis.\n4. Definition of relapsing\u002Frefractory condition: Persistence of disease activity or recurrence of disease activity after remission, despite undergoing conventional treatment for more than six months. Definition of conventional treatment: Administration of glucocorticoids and cyclophosphamide, in combination with any one or more of the following immunomodulatory agents: antimalarial drugs, azathioprine, mycophenolate mofetil, methotrexate, leflunomide, tacrolimus, cyclosporine, as well as biological agents including rituximab, belimumab, telitacicept, etc.\n\nRelapsing and refractory Antiphospholipid Syndrome:\n\n1\\. Meeting the 2006 Sydney-revised diagnostic criteria for primary antiphospholipid syndrome; 2. Testing positive for antiphospholipid antibodies at moderate to high titers (IgG\u002FIgM antibodies against lupus anticoagulant, LA; beta-2 glycoprotein I, B2GP1; or anticardiolipin, acL, detected positive on more than two occasions within a 12-week period); 3. Definition of relapsing\u002Frefractory condition: Recurrence of thrombosis despite standard treatment with warfarin anticoagulation or alternative vitamin K antagonist (i.e., maintaining the required international normalized ratio, INR, for therapeutic management) or standard therapeutic doses of low molecular weight heparin (LMWH), in addition to previous treatment with corticosteroids and cyclophosphamide; 4. For catastrophic antiphospholipid syndrome, the following four criteria must be met: (1) involvement of three or more organs, systems, and\u002For tissues; (2) onset of symptoms within one week; (3) histological confirmation of small vessel occlusion in at least one organ or tissue; (4) presence of aPL (antiphospholipid antibodies).\n\nNote: Meeting either criterion 3 or 4 is sufficient.\n\nRelapsing and refractory Rheumatoid arthritis:\n\n1\\. Diagnosed with rheumatoid arthritis (RA) according to the 2010 American College of Rheumatology (ACR)\u002FEuropean League Against Rheumatism (EULAR) classification criteria, with a history of RA ≥ 3 months; 2. Inadequate response to at least two conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) and at least one biological agent targeting cytokines\u002Fsignal transduction pathways (such as TNFα inhibitors, IL-6R antagonists, anti-CD20 monoclonal antibodies, etc.). (Note: Inadequate response to methotrexate or leflunomide, with stable treatment for ≥ 3 months prior to screening); 3. Moderate to severe active RA: swollen joint count (SJC) ≥ 6, tender joint count (TJC) ≥ 6; 4. Elevation of at least one inflammatory marker: erythrocyte sedimentation rate (ESR) ≥ 28 mm\u002Fh or C-reactive protein (CRP) ≥ upper limit of normal (ULN).\n\nRelapsing and refractory IgG4-Related Disease:\n\n1. Meeting the 2019 American College of Rheumatology (ACR)\u002FEuropean League Against Rheumatism (EULAR) diagnostic criteria for IgG4-related disease (IgG4-RD);\n2. Having an IgG4-RD responder index (RI) ≥ 2 during the screening period, indicating active disease;\n3. Meeting the criteria for refractory or relapsing IgG4-RD:\n\n1\\) Refractory: Defined as a lack of response to steroid therapy or steroid plus immunosuppressive therapy (no clinical or radiological improvement, with a decrease in RI \\\u003C 2); 2) Relapse: Defined as new progression or recurrence of clinical symptoms or radiological manifestations in patients who have previously achieved remission, with or without an elevation in serum IgG4 levels (an increase in RI ≥ 2).\n\nExclusion Criteria:\n\n* Subjects who meet any of the following exclusion criteria will not be admitted to the study:\n\n  1. Individuals with a severe history of drug allergies or those with an allergic constitution;\n  2. Individuals with existing or suspected uncontrolled or treatable fungal, bacterial, viral, or other infections;\n  3. Subjects with central nervous system diseases (excluding those with a history of epilepsy, psychiatric disorders, organic brain disease syndromes, cerebrovascular accidents, encephalitis, or central nervous system vasculitis resulting from the underlying disease);\n  4. Subjects whose cardiac function cannot tolerate the study interventions;\n  5. Subjects with congenital immunoglobulin deficiencies;\n  6. Subjects with a history of malignant tumors within the past five years;\n  7. Subjects with end-stage renal failure;\n  8. Subjects who are positive for hepatitis B surface antigen (HBsAg) and hepatitis B core antibody (HBcAb) with peripheral blood HBV DNA titers exceeding the upper limit of detection; subjects who are positive for hepatitis C virus (HCV) antibody and peripheral blood HCV RNA; subjects who are positive for human immunodeficiency virus (HIV) antibody; and subjects who are positive for syphilis testing;\n  9. Subjects with psychiatric disorders or severe cognitive dysfunction;\n  10. Subjects who have participated in other clinical trials within the past three months prior to enrollment;\n  11. Subjects who have received immunosuppressive agents with therapeutic effects on the disease within five half-lives prior to enrollment or biological agents within four weeks prior to enrollment;\n  12. Pregnant women or women planning to become pregnant;\n  13. Subjects whom the investigator believes have other reasons that preclude their inclusion in this study.","65 Years",{"count":116,"type":21},12,[118],"PHASE1","This study evaluates the safety and efficacy of YTS109 cells in adults with relapsed\u002Frefractory autoimmune diseases, such as Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (SSc), etc. Aproximately 6-12 patients aged 18-65 will receive a single infusion of YTS109 cells (1.5×10⁶ cells\u002Fkg). The main purpose of exploratory clinical research is to explore the efficacy and safety of YTS109 cell and the lymphodepletion regimen. The primary endpoint is observations of types, severity, and frequency of adverse events (AEs) and efficacy assessment. This single-arm, open-label trial will enroll patients across Chinese People's Liberation Army (PLA) General Hospital.",[121,122,123,124,125,72,26,126],"Systemic Lupus Erythematosus (SLE)","Systemic Sclerosis (SSc)","Inflammatory Myopathy","Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis","Antiphospholipid Syndrome (APS)","Sjogren's Syndrome","2025-07-10",{"date":129,"type":43},"2025-07-14",{"date":131,"type":43},"2025-05-19",{"date":133,"type":21},"2027-05-19",{"name":135,"class":136},"China Immunotech (Beijing) Biotechnology Co., Ltd.","INDUSTRY",{"id":138,"slug":139,"hasResults":11,"nctId":140,"briefTitle":141,"officialTitle":142,"acronym":4,"eligibilityCriteria":143,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":114,"enrollmentInfo":144,"targetDuration":4,"studyType":63,"phases":146,"briefSummary":147,"conditions":148,"keywords":4,"overallStatus":39,"whyStopped":4,"lastUpdateSubmitDate":152,"lastUpdatePostDateStruct":153,"startDateStruct":155,"completionDateStruct":157,"leadSponsor":159,"locationsCount":83},"100538011","phase-1-the-bcmacd19-dual-targeted-car-t-cell-in-participants-with-autoimmune-kidney-diseases-100538011","NCT06285279","The BCMA\u002FCD19 Dual Targeted CAR-T Cell in Participants With Autoimmune Kidney Diseases","Evaluation of the Safety and Efficacy of the BCMA\u002FCD19 Dual Targeted CAR-T Cell in Participants With Autoimmune Kidney Diseases: A Single-center Exploratory Clinical Study","Inclusion Criteria:\n\n1. Participants must personally sign an informed consent form approved by the Ethics Committee before the start of the study.\n2. Participants must be aged ≥18 and ≤65 years.\n3. Disease-specific inclusion criteria:\n\n   Active, relapsing, refractory Lupus Nephritis (LN):\n\n   LN diagnosed by kidney biopsy within the last 2 years, with pathological types III, IV, or V, and a chronicity index (CI) score≤3\n\n   Meets one of the following criteria:\n\n   Refractory LN, defined as no remission after at least one standard regimen (CTX and\u002For MMF) for 6 months.\n\n   Relapsing LN, defined as a need to increase steroid dosage to control disease activity during maintenance treatment.\n\n   Clinical criteria: eGFR \\> 45 ml\u002Fmin\u002F1.73 m²; urinary protein quantification ≥ 1.5g\u002F24h; SLE-DAI score ≥ 8.\n\n   ANCA-associated vasculitis (AAV) patients:\n\n   Diagnosed as AAV according to the 2012 Chapel Hill Consensus Conference criteria, meeting one of the following:\n\n   Newly diagnosed AAV with renal involvement:\n\n   Renal involvement must meet both:\n\n   Kidney biopsy showing pauci-immune necrotizing glomerulonephritis. Urinary red blood cells \\>30\u002Fhigh power field.\n\n   Relapsing or refractory AAV:\n\n   Relapse: Defined as an increase in BVAS V3.0 score of ≥1 after remission, requiring adjustment of immunosuppressive treatment to regain remission.\n\n   Refractory: Defined as a) less than 50% reduction in BVAS V3.0 after 6 weeks of standard induction treatment; or b) persistent disease activity (BVAS V3.0 ≥3) after 12 weeks of treatment.\n\n   Membranous nephropathy (MN) patients:\n\n   Tissue biopsy diagnosed as aPLA2R-related membranous nephropathy.\n\n   Clinical criteria for high-risk or relapsing\u002Frefractory membranous nephropathy:\n\n   High-risk patients:\n\n   Defined as meeting any of the following: eGFR normal, urinary protein \\>3.5g\u002Fd, ACEI\u002FARB treatment for 6 months with \\\u003C50% reduction in urinary protein, combined with serum albumin \\\u003C25g\u002Fl or aPLA2R \\>50RU\u002Fml; or eGFR \\\u003C60ml\u002Fmin\u002F1.73m², and\u002For urinary protein \\>8g\u002Fd for over 6 months.\n\n   Refractory\u002Frelapsing membranous nephropathy patients:\n\n   Refractory: Defined as resistance to previous immunosuppressive treatment (persistent urinary protein ≥3.5g\u002Fd with \\\u003C50% reduction compared to baseline).\n\n   Relapse: Defined as complete or partial remission achieved with previous immunosuppressive treatment, followed by reappearance of urinary protein ≥3.5g\u002Fd.\n\n   eGFR ≥ 45 ml\u002Fmin\u002F1.73 m².\n\n   IgG4-related disease patients:\n\n   Meeting the 2019 ACR\u002FEULAR diagnostic criteria for IgG4-related disease, and meeting one of the following:\n\n   Newly diagnosed active IgG4-related disease (Respond Index (RI) ≥3).\n\n   Refractory or relapsed IgG4-related disease:\n\n   Refractory: Defined as no remission with steroid or steroid plus immunosuppressant treatment (no clinical or imaging improvement, RI decrease \\\u003C2) Relapse: Defined as new progression or recurrence of clinical symptoms or imaging findings in a patient who had achieved remission, with or without elevated blood IgG4 (RI increase≥2)\n4. Expected survival ≥ 12 weeks.\n5. ECOG performance status ≤ 2.\n6. Female participants of childbearing potential must agree to use effective contraception from the day of signing the informed consent until 365 days after the infusion. Effective contraception is defined as abstinence or the use of a contraceptive method with a failure rate of \\\u003C1% per year.\n7. Participants must have adequate organ function, meeting all of the following criteria before enrollment:\n\n   1. Absolute neutrophil count ≥ 1.0×10⁹\u002FL \\[Granulocyte colony-stimulating factor (G-CSF) support is allowed, but no supportive treatment should be received within 7 days before the assessment\\].\n   2. Platelet count ≥ 50×10⁹\u002FL \\[No transfusion support (including component transfusion) or treatments aimed\n\nExclusion Criteria:\n\n1. Participants who have received the following previous treatments:\n\n   1.1 Participants who have received gene therapy before enrollment. 1.2 Participants who have been injected with live vaccines within 4 weeks prior to enrollment.\n\n   1.3 Participants who have received other investigational drug treatments within 12 weeks before apheresis.\n2. Participants with active malignancies within the past 5 years, except for tumors deemed curable and cured, such as basal or squamous cell carcinoma, cervical or breast carcinoma in situ, etc.\n3. Participants who are positive for Hepatitis B surface antigen (HBsAg) or Hepatitis B core antibody (HBcAb) with abnormal peripheral blood HBV DNA tests (defined as HBV DNA quantification above the lower limit of detection or above the normal reference range of the testing center, or qualitative HBV DNA test positive); positive for Hepatitis C virus (HCV) antibodies with positive peripheral blood HCV RNA; positive for Human Immunodeficiency Virus (HIV) antibodies; positive for Cytomegalovirus (CMV) DNA; positive for syphilis test RPR.\n4. Participants with uncontrolled active infections (except for \\\u003C Grade 2 CTCAE urinary reproductive system infections and upper respiratory infections).\n5. Participants with severe heart diseases, including but not limited to unstable angina, myocardial infarction (within 6 months prior to screening), congestive heart failure (New York Heart Association \\[NYHA\\] class ≥ III), severe arrhythmias.\n6. Participants with hypertension or diabetes that cannot be controlled with medication.\n7. Participants with unresolved toxic reactions from previous treatments to baseline or ≤ Grade 1 (according to NCI-CTCAE v5.0, except for alopecia and clinically insignificant lab abnormalities).\n8. Participants who have undergone major surgery within 2 weeks prior to enrollment or plan to have surgery during the waiting period for infusion or within 12 weeks after receiving study treatment (except for planned minor surgeries under local anesthesia).\n9. Participants with solid organ transplants.\n10. Pregnant or breastfeeding women.\n11. Participants with a history of central nervous system diseases (such as cerebral aneurysm, epilepsy, stroke, dementia, psychosis, etc.) or consciousness disorders.\n12. Participants with other unstable systemic diseases as judged by the researcher, including but not limited to severe diseases of the liver, kidneys, gastrointestinal tract, or metabolic diseases requiring medication.\n13. Participants are known to have life-threatening allergic reactions, hypersensitivity, or intolerance to FKC288 cellular products or their components.\n14. Participants judged by the researcher to have bleeding, severe thrombosis, or hereditary\u002Facquired bleeding and severe thrombosis conditions (including hemophilia, coagulation dysfunction, thrombocytopenia, splenomegaly, etc.), or patients currently undergoing thrombolytic or anticoagulant therapy.\n15. Participants who have received any B cell-depleting therapy or non-depleting B cell targeted therapy within 6 months.\n16. Participants who have received high-dose methylprednisolone treatment (cumulative dose \\> 1.5g) or cyclophosphamide pulse therapy within a month.\n17. Participants judged by the researcher to be unable to discontinue other immunosuppressants one week before apheresis, or those treated with more than 5 mg\u002Fday of prednisone (or equivalent dose of other corticosteroids).\n18. AAV patients diagnosed with eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) or with active alveolar hemorrhage.\n19. Other conditions deemed by the researcher as unsuitable for enrollment.",{"count":145,"type":21},24,[118],"This study is a single-center, open-label, dose-escalation exploratory clinical trial, expected to enroll 6 to 12 participants. It will use a BOIN (Bayesian Optimal Interval) design for dose escalation, with four predetermined dose groups (0.3×10\\^6 cells\u002Fkg, 1.0×10\\^6 cells\u002Fkg, 3.0×10\\^6 cells\u002Fkg, and an alternative dose of 0.1×10\\^6 cells\u002Fkg). Each dose group plans to enroll 1-2 or 3-6 participants with relapsed or refractory autoimmune-mediated kidney diseases (such as lupus nephritis, ANCA-associated vasculitis, membranous nephropathy, and IgG4-related diseases).",[149,150,151,26],"Lupus Nephritis","ANCA-associated Vasculitis","Membranous Nephropathy - PLA2R Induced","2025-06-06",{"date":154,"type":43},"2025-06-11",{"date":156,"type":43},"2024-03-04",{"date":158,"type":21},"2028-12-31",{"name":160,"class":50},"Nanjing University School of Medicine",{"id":162,"slug":163,"hasResults":11,"nctId":164,"briefTitle":165,"officialTitle":166,"acronym":167,"eligibilityCriteria":168,"healthyVolunteers":169,"sex":170,"minAge":171,"maxAge":4,"enrollmentInfo":172,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":174,"conditions":175,"keywords":187,"overallStatus":39,"whyStopped":4,"lastUpdateSubmitDate":197,"lastUpdatePostDateStruct":198,"startDateStruct":200,"completionDateStruct":202,"leadSponsor":204,"locationsCount":83},"100569575","aylo---autoimmunity-and-loss-of-y-100569575","NCT06696027","AYLo - AutoimmunitY and Loss of y","Investigating the Role of Hematopoietic Mutations and Mosaic Mutation in the Y Chromosome in Autoimmune Rheumatologic Diseases","AYLo","Inclusion Criteria:\n\n* Male\n* \\> 50 years\n* Diagnosis of arthritis (RA, PsA), collagen diseases (SLE, systemic sclerosis, Sjögren's syndrome, mixed connective tissue diseases), vasculitis (eGPA, GPA, MPA, IgG4-related disease, GCA, PMR), sarcoidosis, COPD, ILD or asthma bronchiale confirmed by the treating physician.\n\nExclusion Criteria:\n\n* Female\n* \\\u003C 50 years\n\nInclusion Criteria (Healthy controls):\n\n* Male\n* \\> 50 years\n\nExclusion Criteria (Healthy controls):\n\n* Female\n* \\\u003C 50 years\n* autoimmune, rheumatological diease\n* pulmonary precondition",true,"MALE","50 Years",{"count":173,"type":21},500,"The AYLo study (AutoimmunitY and Loss of y - Investigating the Role of Hematopoietic Mutations and Mosaic Mutation in the Y Chromosome in Autoimmune Rheumatologic Diseases) aims to systematically investigate hematopoietic mutations, such as hematopoietic (mosaic) loss of the Y chromosome (mLOY), focusing on their underlying causes, pathophysiological significance, patterns of manifestation, and impact on disease progression in autoimmune, rheumatologic disorders. This research seeks to bridge existing knowledge gaps by exploring how such mutations influence immune homeostasis, cellular function, and susceptibility to inflammation-driven pathologies.\n\nThrough the integration of advanced immunological profiling, the study aspires to uncover key mechanisms that drive the initiation, progression, and complications of autoimmune rheumatic diseases. These analyses will combine single nucleotide polymorphisms (SNP) arrays, multiplex assays, transcriptomics, and flow cytometry staining of peripheral blood mononuclear cells to delineate the interplay between hematopoietic mutations and immune dysregulation.\n\nA further objective is the development of a multimodal framework for disease-specific characterization, enabling precise mapping of mutation-driven phenotypes across diverse autoimmune conditions. This framework will incorporate clinical, molecular, and imaging data.\n\nAdditionally, the AYLo study aims to explore the potential role of mLOY and other hematopoietic mutations as biomarkers for disease stratification, prognosis, and therapeutic response. The findings may open avenues for personalized treatment approaches, leveraging the molecular insights to inform targeted interventions and improve patient outcomes in autoimmune rheumatic disorders.\n\nBy integrating translational and basic science approaches, this study has the potential to redefine current paradigms in autoimmune disease research and therapy.",[176,177,178,179,26,72,180,181,182,183,184,185,186],"Giant Cell Arteritis (GCA)","Polymyalgia Rheumatica (PMR)","ANCA Associated Vasculitis (AAV)","Idiopathic Inflammatory Myopathies","Psoriatic Arthritis (PsA)","Connective Tissue Disease","Sarcoidosis","Interstitial Lung Disease Due to Systemic Disease (Disorder)","Interstitial Lung Disease (ILD)","Asthma Bronchiale","COPD",[188,189,190,191,192,193,179,26,194,195,181,182,196],"Autoimmune Diseases","Vasculitis","Large Vessel Vasculitis","Giant Cell Arteritis","Polymyalgia Rheumatica","ANCA Associated Vasculitis","Rheumatoid Arthritis","Psoriatic Arthritis","Interstitial Lung Disease","2025-04-07",{"date":199,"type":43},"2025-04-10",{"date":201,"type":43},"2024-11-15",{"date":203,"type":21},"2026-07",{"name":205,"class":50},"University of Bonn",{"id":207,"slug":208,"hasResults":11,"nctId":209,"briefTitle":210,"officialTitle":211,"acronym":4,"eligibilityCriteria":212,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":114,"enrollmentInfo":213,"targetDuration":4,"studyType":63,"phases":215,"briefSummary":217,"conditions":218,"keywords":224,"overallStatus":39,"whyStopped":4,"lastUpdateSubmitDate":228,"lastUpdatePostDateStruct":229,"startDateStruct":231,"completionDateStruct":233,"leadSponsor":235,"locationsCount":83},"100577108","phase-2-bcma-cd19-car-t-therapy-for-refractory-autoimmune-diseases-100577108","NCT06794008","BCMA-CD19 CAR-T Therapy for Refractory Autoimmune Diseases","An Open, Single-Arm, Single-Center Clinical Study Assessing the Safety and Efficacy of BCMA-CD19 Targeted Chimeric Antigen Receptor T-Cell Therapy in Multiple Refractory Autoimmune Diseases","Inclusion Criteria:\n\n1. Age, 18-65 years old (inclusive), weight \\>=45kg, male and female;\n2. The diagnosis of each disease meets the following criteria:\n\nSystemic lupus erythematosus: 1997 ACR classification criteria or 2012 SLICC classification criteria Sjögren's syndrome: 2002 International Classification of Sjögren's Syndrome Inflammatory myopathies: 1977 Bohan Recommendation Systemic sclerosis: 1980 ACR classification criteria or 2013 ACR-EULAR classification criteria Behcet's disease: 1989 International Classification Criteria for Behcet's disease ANCA-associated vasculitis: 1990 ACR classification criteria IgG4-related disease: 2011 IgG4-RD composite diagnostic criteria Antiphospholipid syndrome: 2006 revision of the Sapporo APS classification criteria Acquired thrombotic thrombocytopenic purpura: consistent with a clinical diagnosis of TTP, including microscopic evidence of thrombocytopenia and red blood cell fragmentation (e.g., red blood cell fragmentation) 3. Multiple treatment regimens are ineffective or ineffective (including but not limited to high-dose glucocorticoids, adequate immunosuppressants and biologics) 4. Use of glucocorticoids (\\\u003C=1mg\u002Fkg\u002Fd prednisone or equivalent doses of other hormones), DMARDs (such as methotrexate, hydroxychloroquine, azathioprine, mycophenolate mofetil, leflunomide, cyclosporine, etc.) must be on stable treatment for 4 weeks before receiving the first study drug, and no increase in hormone dose and other immunosuppressants throughout the study.\n\n5\\. Subjects voluntarily participate in this study and voluntarily sign the informed consent form; 6. Subjects who have the possibility of having children or whose partners have the possibility of having children must agree to use effective contraception throughout the study period (but cannot use oral estrogen, use estrogen vaginal ring, etc.) 7. Additional enrollment criteria for different diseases (related to the degree of disease activity):\n\n1. Patients with Behcet's disease must be active patients who meet the following conditions, and the active phase is defined as the emergence of new symptoms or the deterioration of existing symptoms, and one of the following conditions must be met:\n\n   A. Organ involvement: involvement of any major organ (e.g., ocular lesions, vascular lesions, central nervous system, gastrointestinal system); B. 100% increase in the number of oral or genital ulcers \\>=compared to the onset of oral\u002Fgenital ulcers compared to the first day; or an increase in the number of oral or genital ulcers by 3; C. Canker disease is at least 12 months; D. History of several oral ulcers per month E. Arthritis: \\>=50% increase in the number of swollen joints, or 3 more swollen joints; F. Skin lesions (non-oral\u002Fgenital ulcers): \\>= physician overall lesion score increased by \\>=50% or by two points in the total score.\n2. Patients with active inflammatory myopathy need to meet the following additional conditions:\n\n   Active myositis as defined by the Baseline Freehand Muscle Strength Test (MMT-8) of no more than 125\u002F150 and at least 2 additional CSMs that meet the criteria specified below:\n\n   a) Visual Analogue Scale\\[VAS\\] of patient global activity ≥2 cm, b) physician's global disease activity ≥2 cm, c) Health Assessment Questionnaire (HAQ) Disability Index ≥ 0.25 d) Elevation of at least one muscle enzyme \\[including creatine kinase (CK), aldolase, lactate dehydrogenase (LDH), alanine aminotransferase (ALT), and aspartate aminotransferase (AST)\\] with a minimum level of 1.3 x upper limit of normal e) Global Extramuscular Disease Activity Score, with a minimum of 1.0 cm on a 10 cm VAS scale \\[This measure is a physician's comprehensive assessment based on the assessment of physique, skin, bone, gastrointestinal, lung, and cardiac activity scale activity scores using the Myositis Disease Activity Assessment Tool (MDAAT).\n\n   f) To ensure that we are able to recruit patients with active DM with severe rash who may not meet the MMT-8 criteria above, we recommend the use of additional inclusion criteria so that the International Myositis Assessment and Clinical Study (IMACS) Improved Definition (DOI) can be achieved: 1) MDAAT \\> on the 10 cm VAS scale 3 cm skin VAS score, and 2) at least 3 of the above 5 criteria.\n3. ANCA-associated vasculitis:\n\n   A. Comply with GPA\u002FMPA\u002FEGPA classification standards; B. Patients with severe vasculitis activity (meeting at least one of the following conditions);\n\n   a) Renal involvement is characterized by one of the following: i. Evidence of glomerulonephritis in any of the following situations: Renal biopsy shows focal necrotizing glomerulonephritis. Active urinary sediment characterized by glomerular hematuria and proteinuria ii. Patients with prior normal or no prior renal disease document, estimated glomerular filtration rate (eGFR) \\\u003C50 ml\u002Fmin\u002F1.73 m2, and prior chronic kidney disease (eGFR \\\u003C60 ml\u002Fmin\u002F1.73 m2) showed a reduction in eGFR of at least 25% compared with the previous one.\n\n   b) Pulmonary hemorrhage due to active vasculitis satisfies all three of the following: i. Chest X-ray or CT scan showing diffuse pulmonary infiltrates ii. Pulmonary infiltrates that cannot be explained by other causes (e.g., volume overload or pulmonary infection) iii. At least one of the following: Evidence of alveolar hemorrhage on bronchoscopy or bloody bronchoalveolar lavage Hemoptysis was observed Unexplained anemia (\\\u003C10 g\u002FdL) or decreased hemoglobin (\\>1 g\u002FdL) and less than 10g\u002FdL Increased carbon dioxide dispersion\n4. Additional Enrollment Criteria for Systemic Sclerosis:\n\n   Subjects are at high risk of fatal outcomes based on the following prognostic factors: Subjects must have the following \"a\" , and at least one of \"b\" or \"c\".\n\n   a) Diffuse cutaneous scleroderma with an mRSS score of \\>=16, validated by the same physician at 2 different times \\>= 1 day apart and separated by \\\u003C 28 days.\n\n   b) Presence of SSc-related lung disease with FVC \\\u003C 70% or 70% predicted DLCO \\\u003C after hemoglobin correction and evidence of alveolitis obtained by high-resolution chest CT scan or PAL.\n\n   c) History of SSc-related nephropathy, no disease activity before enrollment screening. A history of hypertensive renal crisis with scleroderma is included in this criterion and is defined as follows: i. History of new-onset hypertension based on any of the following (must be repeated and confirmed at least 2 hours apart within 3 days of the first event) with change from baseline SBP\\>=140 mmHg DBP\\>=90 mmHg SBP rose by \\>=30 mmHg compared to baseline DBP increased by \\>=20 mmHg compared to baseline AND ii. One of the following 5 characteristics Serum creatinine increased \\>= \\>50% from baseline proteinuria: \\>=2+; Creatinine ratio \\> upper limit of normal Thrombocytopenia: \\\u003C100, 000 plts\u002Fmm3 Hemolysis: increased by blood smear or reticulocyte count\n5. Additional enrollment criteria for systemic lupus erythematosus A. The SLEDAI score of the patient before enrollment \\>= 7 points B. Failure to receive the following treatments: oral prednisone \\>=20 mg\u002Fd; Cyclophosphamide 0.4 to 0.6 g\u002Fm2 once every two weeks for 6 months, or other immunosuppressants such as mycophenolate mofetil 2 g\u002Fday for 3 months without remission.\n6. Additional enrollment criteria for antiphospholipid syndrome A. Cardiolipin antibody, lupus anticoagulant factor, and anti-β2-glycoprotein 1 antibody were all positive before enrollment.\n\n   B. History of thromboembolism or morbid pregnancy confirmed by clear objective evidence.\n7. Sjögren's disease additional enrollment criteria A. Positive anti-Ro\u002FSSA antibody screen. B. ESSDAI\\>= 6 POINTS\n8. Additional enrollment criteria for IgG4-related diseases (confirmed: A+ B+C) A. Clinical examination showing the presence of characteristic diffuse\u002Flocal swelling or masses in a single or multiple organs.\n\nB. Blood tests show elevated serum IgG4 concentration (135 mg\u002Fdl). C. Histopathological examination shows significant lymphocytic and plasmacytic infiltration and fibrosis or IgG4+ plasmacyte infiltration (IgG4+\u002FIgG+ cell ratio \\>40% and \\>10 IgG4+ plasma cells\u002FHPF).\n\nExclusion Criteria:\n\n1. Use of rituximab or other monoclonal antibodies within 1.6 months.\n2. Received high-dose glucocorticoids (\\>1 mg\u002Fkg\u002Fd) within 1 month.\n3. Serious complications: including heart failure (\\>= NYHA Class III), renal insufficiency (creatinine clearance \\\u003C=30 ml\u002Fmin), hepatic insufficiency (serum ALT or AST greater than three times the upper limit of normal, or total bilirubin greater than the upper limit of normal)\n4. Other severe, progressive, or uncontrollable hematologic, gastrointestinal, endocrine, pulmonary, cardiac, neurological, or cerebral diseases (including demyelinating diseases such as multiple sclerosis).\n5. Known allergies, hyperreactivity, or intolerance to IL-2 or its excipients.\n6. Have a serious infection (including but not limited to hepatitis, pneumonia, bacteremia, pyelonephritis, Epstein-Barr virus, tuberculosis infection), or hospitalization for infection, or use of intravenous antibiotics for treatment of infection 2 months prior to the first dose of treatment.\n7. Chest imaging showing malignancy or current activity within 3 months prior to the first use of study drug Abnormalities in sexually transmitted infections (including tuberculosis).\n8. Infection with HIV (HIV antibody-positive serology) or hepatitis C (Hep C antibody-positive serology).\n\nIf seropositive, it is advisable to consult a physician with expertise in the treatment of HIV or hepatitis C virus infection.\n\n10\\. Any known malignancy or history of malignancy within the past 5 years (with the exception of non-melanoma skin cancer, non-melanoma skin cancer with no signs of recurrence or surgically cured cervical tumor within 3 months prior to the use of the first investigational agent).\n\n11\\. Have an uncontrolled mental or emotional disorder, including a history of drug and alcohol abuse within the past 3 years, which may preclude the successful completion of the study.\n\n12\\. Received or anticipated receipt of any live viral or bacterial vaccine injection within 3 months prior to the first injection of study dose, during the study, or within 4 months after the last injection of study dose. Bacillus Calmette-Guérin vaccination within 12 months of screening.\n\n13\\. Pregnant, lactating women (WCBP) who are unwilling to use medically approved contraception during treatment and for 12 months after the end of treatment.\n\n14\\. Males whose partner is of childbearing potential but who are unwilling to use appropriate medically approved contraception during treatment and for 12 months after the end of treatment.\n\n15\\. Patients with inflammatory myopathies should additionally exclude: 3) Adolescent DM or PM, myositis overlaps with another connective tissue disease, cancer-associated myositis, inclusion body myositis, or any other non-immune-mediated myopathy.\n\n4\\) Severe muscle impairment is defined as a baseline global muscle impairment score of MDI (Myositis Injury Index) \\>=5cm on 10 cm VAS.\n\n16\\. ANCA-associated vasculitis requires additional exceptions: positive anti-GBM antibodies.",{"count":214,"type":21},50,[216],"PHASE2","The objective of this study is to evaluate the efficacy and safety of BCMA\u002FCD19 chimeric antigen receptor (CAR)-modified T cells in the treatment of autoimmune diseases.",[219,123,122,150,26,220,221,222,223],"Systemic Lupus Erythematosus","Antiphospholipid Syndrome","Acquired Thrombotic Thrombocytopenic Purpura","Behcet Disease","Sjogren Syndrome",[225,226,227],"CAR-T","CD19","BCMA","2025-04-01",{"date":230,"type":43},"2025-04-02",{"date":232,"type":43},"2024-12-26",{"date":234,"type":21},"2027-12-31",{"name":236,"class":50},"Peking University People's Hospital",{"id":238,"slug":239,"hasResults":11,"nctId":240,"briefTitle":241,"officialTitle":242,"acronym":4,"eligibilityCriteria":243,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":244,"enrollmentInfo":245,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":247,"conditions":248,"keywords":4,"overallStatus":73,"whyStopped":4,"lastUpdateSubmitDate":249,"lastUpdatePostDateStruct":250,"startDateStruct":252,"completionDateStruct":254,"leadSponsor":256,"locationsCount":4},"100566488","key-mechanisms-of-abnormal-t-cell-activation-and-differentiation-in-igg4-related-ophthalmic-disease-100566488","NCT06655831","Key Mechanisms of Abnormal T Cell Activation and Differentiation in IgG4-Related Ophthalmic Disease","Study on the Key Mechanisms of Abnormal T Cell Activation and Differentiation in IgG4-Related Ophthalmic Disease","Inclusion Criteria:\n\n* Experimental Group: IgG4-ROD patients with lacrimal gland involvement, diagnosed based on 2014 and 2020 Japanese criteria for IgG4-ROD and IgG4-RD:\n\nClinical and imaging features: characteristic diffuse or localized swelling or nodular formations of the lacrimal glands.\n\nSerological diagnosis: Elevated serum IgG4 concentration (\\>135 mg\u002FdL).\n\nHistopathological diagnosis (meeting 2 of the 3 criteria below):\n\nDense lymphoplasmacytic infiltration with fibrosis. IgG4+ plasma cell\u002FIgG+ plasma cell ratio \\>40%, and \\>10 IgG4+ plasma cells per high-power field.\n\nCharacteristic fibrosis, especially storiform fibrosis, or obliterative phlebitis.\n\nControl Group: Patients with lacrimal gland ptosis confirmed by pathology and clinical\u002Fimaging features, requiring surgical repositioning.\n\nExclusion Criteria:\n\n* Presence of other autoimmune diseases like Sjögren's syndrome, systemic lupus erythematosus, or rheumatoid arthritis.\n\nLacrimal gland pathology indicating other orbital diseases such as MALT lymphoma or inflammatory pseudotumor.\n\nOther exclusion criteria include mental disorders, malignancies, or any condition deemed unsuitable for study participation.","80 Years",{"count":246,"type":21},20,"IgG4-related disease (IgG4-RD) is a newly recognized chronic inflammatory condition caused by immune system dysfunction. It is characterized by the infiltration of IgG4+ plasma cells, dense fibrosis, and inflammation involving veins and eosinophils. Common symptoms include elevated serum IgG4 levels and the formation of tumor-like growths that can affect almost any organ, leading to pressure on tissues, irreversible damage, and even organ failure. While estimates suggest a prevalence of 0.28 to 1.08 per 100,000 people, this might be an underestimation due to limited awareness, the new definition of the disease, and its subtle onset.\n\nIgG4-related ophthalmic disease (IgG4-ROD) is a subtype of IgG4-RD that affects the eye area, particularly the lacrimal glands, extraocular muscles, and surrounding nerves. It often presents as painless swelling of the lacrimal glands in one or both eyes, sometimes with discomfort or a sensation of a foreign body. It can also cause thickening of eye muscles, leading to symptoms like bulging eyes, blurred vision, or double vision. In some cases, mass lesions in the orbit may press on the optic nerve, potentially leading to permanent vision loss. While there is currently no cure for IgG4-ROD, steroids are used as the main treatment to control inflammation and fibrosis. However, the disease often recurs, with recurrence rates for IgG4-RD reported between 24% and 63% in various studies. Understanding the causes of IgG4-ROD could help develop better treatments and reduce the chances of relapse.\n\nStudies suggest that T cells play a key role in the development of IgG4-RD, including IgG4-ROD. CD4+ T cells are the main immune cells found in affected tissues. They can help B cells multiply and produce IgG4 antibodies and contribute to tissue fibrosis by releasing certain signaling molecules. Despite treatment with rituximab, a drug that targets B cells, many IgG4-RD patients experience relapses, indicating that T cells remain important in driving the disease. Among the T cell subtypes, T follicular helper cells (Tfh) and CD4+ cytotoxic T cells (CD4+ CTLs) are particularly relevant. Tfh cells support B cells in producing IgG4 antibodies, while CD4+ CTLs can contribute to tissue fibrosis by releasing factors like TGF-β, IL-1β, and IFN-γ. However, the detailed mechanisms of how T cells become abnormally activated and differentiated in IgG4-ROD remain unclear.\n\nThis study will use samples from the lacrimal glands, blood, and tears of IgG4-ROD patients to investigate how T cells become abnormally active and differentiate in this condition. The findings could identify new targets for therapy, helping to reduce the recurrence of IgG4-ROD and provide insights into treating other forms of IgG4-RD.",[26],"2024-10-21",{"date":251,"type":43},"2024-10-23",{"date":253,"type":21},"2024-11-01",{"date":255,"type":21},"2025-02-01",{"name":257,"class":50},"Peking University Third Hospital"]