[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"common-variable-immunodeficiency\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:common-variable-immunodeficiency":28},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,7,0,[8,47,82,92,114,137,165],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":13,"acronym":4,"eligibilityCriteria":14,"healthyVolunteers":15,"sex":16,"minAge":17,"maxAge":18,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":23,"conditions":24,"keywords":29,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":37,"lastUpdatePostDateStruct":38,"startDateStruct":41,"completionDateStruct":4,"leadSponsor":43,"locationsCount":46},"100053333","natural-history-of-bronchiectasis-100053333",false,"NCT00943514","Natural History of Bronchiectasis","* PARTICIPANT INCLUSION CRITERIA:\n\n  1. Children age 5 years and above and adults referred to the NIH with chronic or recurring respiratory infections will be eligible for participation.\n  2. Males and females will be considered without regard to race or ethnicity or upper age limit.\n  3. Emphasis will be primarily on non CF bronchiectasis for elucidation of mechanisms of infection susceptibility. However, select patients with cystic fibrosis or acquired immune defects (such as HIV) may be studied if relevant host defects are suspected, if needed for comparison purposes, or to evaluate and study the management of chronic pathogen associated conditions such as nontuberculous mycobacterial infections or allergic bronchopulmonary aspergillosis.\n  4. Subjects must have a primary or referring physician\n  5. Subjects must be willing to have samples stored\n\nINCLUSION CRITERIA FOR RELATIVES:\n\nAs a part of this protocol we may obtain blood, sputum, urine, or buccal swabs from some blood relatives of patients on the study, with the hope of isolating and characterizing the primary host defense defect(s) or genetic links responsible for airway infection susceptibility and\u002For bronchiectasis seen within families. Male and female relatives will be accepted without limitation due to age. These relatives may have pertinent disease-related history obtained, but will neither receive treatment nor have any other protocol procedures done unless they are enrolled on the study.\n\nPARTICIPANT EXCLUSION CRITERIA:\n\n1. We wish to avoid enrolling subjects, especially children, who have common respiratory problems (aeroallergen sensitivity, asthma, gastric esophageal reflux) that are not associated with an underlying abnormality in host defenses. Evidence of significance of chronic or recurring infections suggestive of an underlying airway surface or systemic host defense defect should be documented by one or more of the following: a) sinus or lower airway cultures positive for bacterial, fungal, or mycobacterial pathogens characteristic of these defects; or b) radiographic evidence of sinusitis with mucosal thickening and\u002For air-fluid levels; or c) radiographic evidence of bronchiectasis; d) severity of otitis media requiring placement of tympanic membrane pressure equalization tubes; e) severity of sinusitis requiring surgical intervention.\n2. Patients who are unable or unwilling to provide informed consent either directly or via appropriately designated surrogate.\n3. Any patient who, in the opinion of the Investigator, is unable or unwilling to comply with regular follow-up or is unlikely to provide pertinent information regarding disease progression or response to treatment may be excluded from longitudinal follow-up.\n\nPARTICIPATION OF CHILDREN:\n\nChildren under the age of 5 will be excluded from this protocol due to the difficulty of performing pertinent assays in infants and younger children, difficulty distinguishing significance of respiratory infections which occur commonly in younger children, and the lack of adequate facilities and equipment for management of children younger than 2 years.",true,"ALL","5 Years","100 Years",{"count":20,"type":21},900,"ESTIMATED","OBSERVATIONAL","Background:\n\n* Bronchiectasis is a disease characterized by airways that are inflamed, abnormally dilated, and chronically infected. Individuals with bronchiectasis have a history of chronic and recurring respiratory infections. Depending on the underlying cause, these infections may involve the entire respiratory tract, resulting in sinus, ear, and lung disease.\n* Bronchiectasis continues to be a significant problem in developing countries and in specific groups of individuals, particularly in people who have cystic fibrosis. Although treatments are available or under development for bronchiectasis related to cystic fibrosis, many of the disease-specific treatments may not be effective for bronchiectasis not related to cystic fibrosis.\n\nObjectives:\n\n\\- To study the natural history of bronchiectasis to identify inherited and immune factors that may explain why certain individuals have chronic recurring infections.\n\nEligibility:\n\n* Individuals 5 years of age and older who have an established diagnosis of bronchiectasis or a history of chronic\u002Frecurring respiratory infections.\n* Direct family members (e.g., parents, siblings, children) of patients in the study may also be asked to participate.\n\nDesign:\n\n* Potential participants will be screened with an initial clinic evaluation and full medical history, as well as a general quality of life and respiratory symptom questionnaire.\n* The following standard procedures may be done as part of the study: air sampling from the nose; imaging studies, which may include an x-ray or computed tomography (CT), lung function tests; and collection of samples of blood, urine, and sputum (phlegm or mucus). Other tests may be performed as required by the researchers, and will be explained to patients as needed.\n* Both patients and relatives (if asked to participate) will provide the following samples: blood or buccal (cheek swab) cells for genetic testing, sputum, and urine.\n* To prevent infections and potential disease progression, patients may receive standard medical care and treatment for bronchiectasis and related infections during this protocol.",[25,26,27,28],"Bronchiectasis","Cystic Fibrosis","Autoimmune Disease","Common Variable Immunodeficiency",[30,31,32,33,34,35],"Variant Cystic Fibrosis","Bronchopulmonary Mycosis","Airway Infection Susceptibility","Chronic Respiratory Infection","Primary Ciliary Dyskinesia","Natural History","RECRUITING","2026-07-10",{"date":39,"type":40},"2026-07-13","ACTUAL",{"date":42,"type":40},"2009-08-01",{"name":44,"class":45},"National Heart, Lung, and Blood Institute (NHLBI)","NIH",1,{"id":48,"slug":49,"hasResults":11,"nctId":50,"briefTitle":51,"officialTitle":51,"acronym":4,"eligibilityCriteria":52,"healthyVolunteers":15,"sex":16,"minAge":53,"maxAge":54,"enrollmentInfo":55,"targetDuration":4,"studyType":57,"phases":58,"briefSummary":60,"conditions":61,"keywords":66,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":72,"lastUpdatePostDateStruct":73,"startDateStruct":75,"completionDateStruct":77,"leadSponsor":79,"locationsCount":81},"100253594","phase-2-pilot-trial-of-allogeneic-blood-or-marrow-transplantation-for-primary-immunodeficiencies-100253594","NCT02579967","Pilot Trial of Allogeneic Blood or Marrow Transplantation for Primary Immunodeficiencies","* INCLUSION CRITERIA - RECIPIENT:\n* Patients age \\>= 4 through 75 years\n* PID deemed to be of sufficient past severity to warrant allo BMT, by meeting the two criteria below:\n\n  1. PID as defined by identified genetic defect or, in the absence of a PID-associated genetic mutation, patients with an immune defect potentially amenable to allo BMT who meet the clinical history criteria below may be eligible upon discussion with the PI\n\n     * Mutations should be confirmed in a CLIA-certified laboratory, if such testing is available.\n     * Patients without a mutation must be deemed eligible and appropriate for allo BMT by the PI. Some patients may meet the clinical history criteria listed below, but will not be eligible if it is thought that their clinical history is due to a condition apart from an immune defect. In addition, patients with a PID of mild severity, such as those with selective IgA deficiency, may meet at least two of the clinical history criteria, but may be deemed inappropriate for allo BMT by the PI if it is felt that the risks of the procedure outweigh the severity of the disease.\n  2. Clinical history of at least two of the following:\n\n     * Life-threatening, organ-threatening, or severely disfiguring infection\n     * Protracted or recurrent infections requiring unusually long or repeated courses of antibiotics\n     * Infection with an opportunistic organism\n     * Chronic elevation in the blood (\\>=2 documented elevations over a period of 6 months or longer) of a latent virus (EBV, CMV, HHV6, HHV8, etc.)\n     * Evidence of immune dysregulation, as manifested by autoimmune disease, atopy, hemophagocytic lymphohistiocytosis\u002Fmacrophage activation syndrome, granulomas, splenomegaly, or lymphadenopathy\n     * Patients with hemophagocytic lymphohistiocytosis or macrophage activation syndrome related to an underlying lymphoma with no other clinical history suggestive of a primary immunodeficiency will not be eligible\n     * Hypogammaglobulinemia, dysglobulinemia, or impaired response to vaccination\n     * Hematologic malignancy or lymphoproliferative disorder\n     * Tissue diagnosis should be confirmed by NCI Department of Pathology, if prior biopsies are available\n     * Virus-associated solid tumor malignancy or pre-cancerous lesion\n     * Tissue diagnosis should be confirmed by NCI Department of Pathology, if prior biopsies are available\n* Availability of at least one 7-8\u002F8 (9-10\u002F10) HLA-matched related (excluding an identical twin) or unrelated donor, or an HLA-haploidentical related donor\n* Consensus among the PI, key AIs, and consultants (as necessary) that correction of the patient s immune system through BMT has the potential to improve the patient s health, quality of life, and\u002For life expectancy, after taking into consideration the patient s existing non-hematopoietic, potentially irreversible organ dysfunction\n* Adequate end-organ function, as measured by:\n\n  * Left ventricular ejection fraction (LVEF) \\>= 40% by 2D echocardiogram (ECHO) or MUGA, or left ventricular shortening fraction \\>= 20% by ECHO for patients receiving RIC or RIC-MMF, or RIC-SHORT, or LVEF \\>= 30% if the patient has radiologic evidence of aortic, renal, or coronary artery vasculitis.\n  * Pulmonary function tests: DL(co) (corrected for hemoglobin) and FEV(1) \\>= 40% of predicted for the RIC, RIC-MMF, and RIC-SHORT arms; or in pediatric patients, if unable to perform pulmonary function tests, there should be no evidence of dyspnea at rest, no requirement for supplemental oxygen, and oxygen saturation \\>92% on room air. Calculations will be based on the values reported in CRIS.\n  * Bilirubin \\\u003C= 3.0 mg\u002FdL (unless due to Gilbert s syndrome or hemolysis) for patients receiving RIC, RIC-MMF, RIC-SHORT; ALT and AST 10 x ULN for patients receiving RIC, RIC-MMF, RIC-SHORT. Patients who are above these bilirubin, ALT, or AST thresholds may be eligible for the RIC, RIC-MMF, or RIC-SHORT arms if evaluated by a hepatologist who deems the liver function test abnormalities to be potentially reversible with bone marrow transplant.\n  * Estimated creatinine clearance of \\>= 40 mL\u002Fmin\u002F1.73 m\\^2, calculated using the Cockcroft-Gault equation for adults and Schwartz formula for pediatric patients, for patients with creatinine levels above the institutional upper limit of normal\n* Karnofsky or Lansky performance status of \\>=60% or ECOG performance status of 2 or less\n* Ability of subject or Legally Authorized Representative (LAR) to understand and the willingness to sign a written informed consent document\n* Not pregnant or breastfeeding. As therapeutic agents used in this trial may be harmful to a fetus, women of childbearing potential and men must agree to use adequate contraception (hormonal or barrier method of birth control; abstinence) prior to study entry and for at least one year post-allo BMT. Should a woman become pregnant or suspect she is pregnant while she or her partner is participating in the study, she should inform her treating physician immediately.\n* Disease status: Patients with malignancy are to be referred in remission for evaluation, except in cases of virus-associated malignancy who may be referred at any time. Should a patient have progressive disease or a donor becomes unavailable after enrollment, the patient will be referred back to his\u002Fher primary hematologist-oncologist for treatment. If this course of action is not in the best interest of the patient according to the clinical judgment of the PI, then the patient may receive standard treatment for the malignant disease under the current study, although this should only occur as a bridge to transplant. If under either of these settings, it becomes apparent that the patient will not be able to proceed to transplant, then he\u002Fshe must come off the study. Patients receiving standard therapy will be told about the therapy, associated risks, potential benefits, alternatives to the proposed therapy, and the availability of receiving the same treatment elsewhere, outside of a research protocol.\n\nEXCLUSION CRITERIA - RECIPIENT:\n\n* Patients who are receiving any other investigational agents, with the exception of virus-specific cytotoxic T-cells for the treatment of viral infection\u002Freactivation prior to allo BMT.\n* History of allergic reactions attributed to compounds of similar chemical or biologic composition to agents (cyclophosphamide, busulfan, pentostatin, sirolimus, MMF, filgrastim or filgrastim biosimilar) used in the study\n* Active psychiatric disorder which may compromise compliance with the transplant protocol, or which does not allow for appropriate informed consent\n* Active central nervous system (CNS) involvement by malignancy, except in cases of virus-associated malignancies with CNS involvement in which case the patient may benefit from the transplant to control the malignancy.\n* MAGT1 mutation and active need to take anti-platelet agents and\u002For therapeutic anti-coagulation that cannot be interrupted during aplasia.\n* HIV positive or other acquired immunodeficiency that, as determined by the PI, interferes with the assessment of PID severity and\u002For the attribution of clinical manifestations of immunodeficiency to a PID.\n* Lack of adequate central venous access potential\n\nInclusion Criteria (Related Donor):\n\n* Ages \\>= 4\n* Related donor deemed suitable and eligible and willing to donate per clinical evalations who are additionally willing to donate blood, urine, and marrow specimens for research. Related donors will be evaluated in accordance with existing Standard Policies and Procedures for determination of eligibility and suitability for clinical donation. Note that participation in this study is offered to all related donors but is not required for clinical donation, so it is possible that not all related donors will enroll on this study.\n\nExclusion Criteria (Related Donor):\n\nNone\n\nINCLUSION CRITERIA - UNRELATED DONOR:\n\n* Ages \\>= 18\n* Unrelated donors will be evaluated in accordance with existing NMDP Standard Policies and Procedures, available at: http:\u002F\u002Fbethematch.org\u002FAbout-Us\u002FGlobal-transplant-network\u002FStandards\u002F, except for the additional requirement of EBV serostatus testing. Note that participation in this study is offered to all unrelated donors but not required for clinical donation, so it is possible that not all unrelated donors will enroll on this study.\n\nEXCLUSION CRITERIA - UNRELATED DONOR:\n\n-Unrelated donors: failure to qualify as a National Marrow Donor Program (NMDP) donor per current NMDP Standards, available at: http:\u002F\u002Fbethematch.org\u002FAbout-Us\u002FGlobal-transplant-network\u002FStandards\u002F.","4 Years","75 Years",{"count":56,"type":21},354,"INTERVENTIONAL",[59],"PHASE2","Background:\n\nAllogeneic blood or marrow transplant is when stem cells are taken from one person s blood or bone marrow and given to another person. Researchers think this may help people with immune system problems.\n\nObjective:\n\nTo see if allogeneic blood or bone marrow transplant is safe and effective in treating people with primary immunodeficiencies.\n\nEligibility:\n\nDonors: Healthy people ages 4 or older\n\nRecipients: People ages 4-75 with a primary immunodeficiency that may be treated with allogeneic blood or marrow transplant\n\nDesign:\n\nParticipants will be screened with medical history, physical exam, and blood tests.\n\nParticipants will have urine tests, EKG, and chest x-ray.\n\nDonors will have:\n\nBone marrow harvest: With anesthesia, marrow is taken by a needle in the hipbone.\n\nOR\n\nBlood collection: They will have several drug injections over 5-7 days. Blood is taken by IV in one arm, circulates through a machine to remove stem cells, and returned by IV in the other arm.\n\nPossible vein assessment or pre-anesthesia evaluation\n\nRecipients will have:\n\nLung test, heart tests, radiology scans, CT scans, and dental exam\n\nPossible tissue biopsies or lumbar puncture\n\nBone marrow and a small piece of bone removed by needle in the hipbone.\n\nChemotherapy 1-2 weeks before transplant day\n\nDonor stem cell donation through a catheter put into a vein in the chest or neck\n\nSeveral-week hospital stay. They will take medications and may need blood transfusions and additional procedures.\n\nAfter discharge, recipients will:\n\nRemain near the clinic for about 3 months. They will have weekly visits and may require hospital readmission.\n\nHave multiple follow-up visits to the clinic in the first 6 months, and less frequently for at least 5 years.",[62,28,63,64,65],"Primary T-cell Immunodeficiency Disorders","Immune System Diseases","Autoimmune Lymphoproliferative","Lymphoproliferative Disorders",[67,68,69,70,71],"Haploidentical","Autoimmunity","Immune Dysregulation","Congenital","Opportunistic Infection","2026-06-27",{"date":74,"type":40},"2026-06-30",{"date":76,"type":40},"2015-11-19",{"date":78,"type":21},"2036-12-31",{"name":80,"class":45},"National Cancer Institute (NCI)",2,{"id":83,"slug":4,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":13,"acronym":4,"eligibilityCriteria":14,"healthyVolunteers":15,"sex":16,"minAge":17,"maxAge":18,"enrollmentInfo":84,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":23,"conditions":85,"keywords":86,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":87,"lastUpdatePostDateStruct":88,"startDateStruct":90,"completionDateStruct":4,"leadSponsor":91,"locationsCount":46},"100128676",{"count":20,"type":21},[25,26,27,28],[30,31,32,33,34,35],"2026-06-18",{"date":89,"type":40},"2026-06-22",{"date":42,"type":40},{"name":44,"class":45},{"id":93,"slug":94,"hasResults":11,"nctId":95,"briefTitle":96,"officialTitle":97,"acronym":4,"eligibilityCriteria":98,"healthyVolunteers":11,"sex":16,"minAge":53,"maxAge":99,"enrollmentInfo":100,"targetDuration":4,"studyType":57,"phases":102,"briefSummary":103,"conditions":104,"keywords":105,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":106,"lastUpdatePostDateStruct":107,"startDateStruct":109,"completionDateStruct":111,"leadSponsor":113,"locationsCount":46},"100388586","phase-2-allogeneic-hematopoietic-stem-cell-transplant-for-patients-with-inborn-errors-of-immunity-100388586","NCT04339777","Allogeneic Hematopoietic Stem Cell Transplant for Patients With Inborn Errors of Immunity","A Phase II Study of Allogeneic Hematopoietic Stem Cell Transplant for Patients With Inborn Errors of Immunity","* INCLUSION CRITERIAl:\n* Age \\>= 4 years and \\\u003C=69 yo with Weight \\>=12 kilograms\n* Mutation in a known monogenic (IEI) gene performed by a CLIA certified laboratory, who have failed standard medical management, or when no standard medical management is available.\n\nOR\n\nPatients without a known IEI mutation may be eligible if they have a clinical history that is characteristic of an individual with an immune defect including a history of infections requiring prolonged courses of therapy or evidence of immune dysregulation manifested by autoimmune\u002Fautoinflammatory disease, atopy, hemophagocytic lymphohistiocytosis, hypogammaglobulinemia, or impaired response to vaccination. A virally-driven malignancy alone will also constitute basis for inclusion.\n\n* Availability of an 8\u002F8, 7\u002F8, or 6\u002F8 HLA-matched related or unrelated donor (if the mismatch is at DQ this will be considered an 8\u002F8 matched donor), or a haploidentical related donor. Karnofsky or Lansky performance status of \\>= 40%\n* Adequate end-organ function, as measured by:\n\n  --Left ventricular ejection fraction \\> 40%, preferably by 2-D echocardiogram (ECHO) obtained within 60 days prior to enrollment.\n* Creatinine: Adult patients: \\\u003C= 2.0 mg\u002Fdl and creatinine clearance \\>= 30 ml\u002Fmin; Pediatric patients (\\\u003C18 years old): creatinine \\\u003C 1.5 mg\u002FdL and a creatinine clearance, using the Schwartz Formula \\> 30 mL\u002Fmin\u002F1.73m\\^2.\n\n  * Serum conjugated bilirubin \\\u003C 2.5 mg\u002Fdl; serum ALT and AST \\\u003C= 5 times upper\n\nlimit of normal.\n\n--Pulmonary function tests: FEV1 \\> 30% and DLCO \\>30%. Children who are unable to have DLCO assessed due to age, are still eligible if no evidence of dyspnea at rest and no need for supplemental oxygen.\n\n* Ability of subject or parent\u002Fguardian to understand and the willingness to sign a written informed consent document. For subjects \\\u003C18 years old, their legal guardian must give informed consent. Pediatric patients will provide assent.\n* As therapeutic agents used in this trial may be harmful to a fetus, women of childbearing potential and men must agree to use adequate contraception (hormonal or barrier method of birth control; abstinence) prior to study entry and for at least one year post-allo HCT. Should a woman become pregnant or suspect she is pregnant while she or her partner is participating in the study, she should inform her treating physician immediately.\n* Willingness to remain in the NIH hospital or, if discharged, stay close to the NIH, for a minimum of 100 days after transplant or longer, if there are complications. If outpatient in the first 100 days after transplant, patient must commit to having an adult caregiver with them at all times.\n\nEXCLUSION CRITERIA:\n\n* Patients who are receiving any other investigational agents (with the exception of virus-specific therapy e.g. cytotoxic T-cells for the treatment of viral infection\u002Freactivation prior to allo HCT).\n* Patients with known brain metastases should be excluded from this clinical trial because of their poor prognosis and because they often develop progressive neurologic dysfunction that would confound the evaluation of neurologic and other adverse events.\n* HIV-positive patients are ineligible because these patients are at increased risk of lethal infections when treated with marrow-suppressive therapy. Appropriate studies will be undertaken in patients receiving combination antiretroviral therapy when indicated.\n* History of allergic reactions attributed to compounds of similar chemical or biologic composition to agents (steroids, cyclophosphamide, busulfan, tacrolimus, sirolimus, MMF, G-CSF, alemtuzumab) used in the study\n* Active psychiatric disorder which is deemed by the PI to have significant risk of compromising compliance with the transplant protocol or which does not allow for appropriate informed consent\n* Pregnant women are excluded from this study because the study agents have the potential for teratogenic or abortifacient effects. Because there is an unknown but potential risk for adverse events in nursing infants secondary to treatment of the mother with the study agents, breastfeeding should be discontinued if the mother is treated with the study agents.\n* Uncontrolled intercurrent illness including, but not limited to, symptomatic congestive heart failure, unstable angina pectoris, cardiac arrhythmia, or psychiatric illness\u002Fsocial situations that would limit compliance with study requirements.","69 Years",{"count":101,"type":21},66,[59],"Background:\n\nDuring a transplant, blood stem cells from one person are given to someone else. The cells grow into the different cells that make up the immune system. This can cure people with certain immunodeficiencies. But transplant has many risks and complications.\n\nObjective:\n\nTo see if stem cell transplant can be successfully performed in people with primary immunodeficiency disease and cure them.\n\nEligibility:\n\nPeople ages 4-69 for whom a primary immunodeficiency (PID) or Primary Immune Regulatory Disorder (PIRD), has caused significant health problems and either standard management has not worked or there are no standard management options, along with their donors\n\nDesign:\n\nDonors will be screened under protocol 01-C-0129. They will donate blood or bone marrow.\n\nParticipants will be screened with:\n\nMedical history\n\nPhysical exam\n\nBlood, urine, and heart tests\n\nCT or PET scans\n\nBefore transplant, participants will have dental and eye exams. They will have a bone marrow biopsy. For this, a needle will be inserted through the skin into the pelvis to remove marrow.\n\nParticipants will be hospitalized before their transplant. They will have a central catheter put into a vein in their chest or neck. They will get medications through the catheter to prevent complications. Participants will get stem cells through the catheter. They will stay in the hospital for at least 4 weeks. They will give blood, urine, bone marrow, and stool samples. They may need blood transfusions. They may need more scans. They will take more medications.\n\nParticipants will have visits on days 30, 60, 100, 180, and 360, and 24 months after the transplant. Then they will have visits once a year for about 5 years",[65,64,63,28,62],[68,67,71,69,70],"2026-05-01",{"date":108,"type":40},"2026-05-04",{"date":110,"type":40},"2020-09-22",{"date":112,"type":21},"2027-11-30",{"name":80,"class":45},{"id":115,"slug":116,"hasResults":11,"nctId":117,"briefTitle":118,"officialTitle":119,"acronym":4,"eligibilityCriteria":120,"healthyVolunteers":15,"sex":16,"minAge":121,"maxAge":4,"enrollmentInfo":122,"targetDuration":4,"studyType":57,"phases":124,"briefSummary":126,"conditions":127,"keywords":4,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":128,"lastUpdatePostDateStruct":129,"startDateStruct":130,"completionDateStruct":132,"leadSponsor":134,"locationsCount":46},"100454131","phase-4-usage-of-spirometry-in-managing-igg-therapy-in-cvid-with-airway-disease-100454131","NCT05193552","Usage of Spirometry in Managing IgG Therapy in CVID With Airway Disease","A Prospective Study of the Utility of Spirometry to Identify and Manage Immunoglobulin Replacement Dosage in Primary Antibody Deficiency in Patients With Potentially Reversible Airway Disease","Inclusion Criteria:\n\n1. Patients who meet criteria for common variable immune deficiency (CVID) who are on stable IGRT for at least 3 months and who have an FEF25-75% between 50% and 80% of predicted.\n2. Patients who are already on Hizentra will be preferred.\n\nExclusion Criteria:\n\n1. Age \\\u003C21 or cannot perform spirometry.\n2. Smokers with 20 pack years or more, and active smokers will not be included among the study subjects, but will be considered separately as an ancillary study.\n3. Patients with specific antigen-specific antibody deficiencies or X-linked agammaglobulinemia on IGRT will not be included among the 20 study subjects, but will be considered separately in ancillary studies.\n4. Patients with heart failure, TB, bronchiolitis, or lymphangioleiomyomatosis.","21 Years",{"count":123,"type":21},22,[125],"PHASE4","Although there is evidence in the literature that gammaglobulin replacement therapy can lead to a reduction in the prevalence of pulmonary infection and improved lung function, there is no published study to guide immunologists regarding the use of spirometry in titrating IG therapy to assist in the management of immunodeficiency patients with regards to gammaglobulin replacement therapy.\n\nThe investigators propose to study the use of spirometry to identify patients that could potentially benefit from an increase in IGRT. The investigators will identify 22 common variable immune deficiency (CVID) study subjects on stable IGRT replacement therapy equivalent to 0.40 to 0.60 gm\u002Fkg per 4 weeks who have evidence of mild to moderate obstruction as assessed by an FEF25-75% between 50% and 80% of predicted. Patients who are on Hizentra will be preferentially recruited. Of these 22, 11 will be identified at random and treated for 6 months at their current dose (control population). The remaining 11 study subjects (treatment group) will have their level of IGRT increased by the equivalent of 0.05 gm\u002Fkg in dose per 4 weeks, adjusted for bioavailability as per manufacturer's instructions. On average, rounded up to the nearest gram, this will typically increase their dose of Hizentra by 2 gm per week.",[28],"2026-04-28",{"date":108,"type":40},{"date":131,"type":40},"2024-01-15",{"date":133,"type":21},"2027-12",{"name":135,"class":136},"University of Alabama at Birmingham","OTHER",{"id":138,"slug":139,"hasResults":11,"nctId":140,"briefTitle":141,"officialTitle":142,"acronym":143,"eligibilityCriteria":144,"healthyVolunteers":15,"sex":16,"minAge":145,"maxAge":4,"enrollmentInfo":146,"targetDuration":4,"studyType":57,"phases":148,"briefSummary":150,"conditions":151,"keywords":152,"overallStatus":156,"whyStopped":4,"lastUpdateSubmitDate":157,"lastUpdatePostDateStruct":158,"startDateStruct":160,"completionDateStruct":162,"leadSponsor":163,"locationsCount":46},"100612559","peripheral-helper-t-cells-in-common-variable-immunodeficiency-100612559","NCT07255157","Peripheral Helper T-cells in Common Variable ImmunoDeficiency","Deciphering the Role of Peripheral Helper T-cells in Common Variable ImmunoDeficiency Pathophysiology in Patients With Non-infectious Complications","TAPDI","Inclusion Criteria:\n\n* Male or female;\n* Age ≥ 18 years;\n* Standard criteria will be applied to diagnose CVID, specifically requiring: 1) low serum IgG level \\\u003C5 g\u002FL, combined with low IgM- and\u002For IgA-isotype concentrations \\\u003C0.4 g\u002FL or \\\u003C0.7 g\u002FL, respectively; 2) poor antibody responses to immunization or infection; and 3) exclusion of other defined forms of secondary hypogammaglobulinemias. Patients meeting the definitional criteria for CVID will be included, regardless of the duration of the disease or the treatment received (gammaglobulins substitution or not);\n* Being affiliated to health insurance;\n* Willing to participate and to sign informed consent.\n\nExclusion Criteria:\n\n* Patients on corticosteroids and\u002For immunosuppressants;\n* Patients with a primary immunodeficiency genetically characterized, such as Bruton disease our HyperIgM syndrome;\n* Patients with an active chronic infection;\n* Pregnant or breastfeeding women;\n* Persons deprived of their liberty by a judicial or administrative decision, minors, persons of legal age who are the object of a legal protection measure or unable to express their consent.","18 Years",{"count":147,"type":21},60,[149],"NA","The aim is to determine whether whether Tph could support non-infectious complications through providing help to pathological B-cells.",[28],[153,154,155],"common variable immunodeficiency","peripheral helper T cells","B cells","NOT_YET_RECRUITING","2025-12-02",{"date":159,"type":40},"2025-12-10",{"date":161,"type":21},"2025-12",{"date":133,"type":21},{"name":164,"class":136},"University Hospital, Bordeaux",{"id":166,"slug":167,"hasResults":11,"nctId":168,"briefTitle":169,"officialTitle":169,"acronym":170,"eligibilityCriteria":171,"healthyVolunteers":11,"sex":16,"minAge":53,"maxAge":4,"enrollmentInfo":172,"targetDuration":4,"studyType":57,"phases":174,"briefSummary":175,"conditions":176,"keywords":4,"overallStatus":36,"whyStopped":4,"lastUpdateSubmitDate":178,"lastUpdatePostDateStruct":179,"startDateStruct":181,"completionDateStruct":183,"leadSponsor":185,"locationsCount":187},"100433534","phase-2-abatacept-for-the-treatment-of-common-variable-immunodeficiency-with-interstitial-lung-disease-100433534","NCT04925375","Abatacept for the Treatment of Common Variable Immunodeficiency With Interstitial Lung Disease","ABCVILD","Inclusion Criteria:\n\n1. Diagnosis of CVID according to the international consensus document (ICON)\n\n   1. Age 4 years or above\n   2. Serum IgG at least 2 standard deviations below the age adjusted normal\n   3. Decreased serum IgA and\u002For serum IgM\n   4. Abnormal specific antibody response to immunization\n   5. Exclusion of secondary immunodeficiency\n2. On replacement immunoglobulin for at least 6 months and willing to maintain throughout study\n3. Granulomatous-lymphocytic interstitial lung disease with a lymphocytic component diagnosed by lung biopsy prior to study entry, wedge biopsy preferred.\n4. Persistence or worsening of interstitial lung disease measured on serial CT imaging of the lung at least 6 months apart, with the latest assessment within 3 months of study entry.\n5. Signed written informed consent\n6. Willing to allow storage of biological specimens for future use in medical research.\n7. Female subjects of childbearing potential must agree to an effective form of birth control such as hormone based contraceptive, intrauterine device, condoms\u002Fbarrier, surgically sterile partner, or abstinence.\n8. Fertile, non-vasectomized males with a female partner of childbearing potential should use condoms throughout the study and for 3 months after the last dose\n\nExclusion Criteria:\n\n1. History of hypersensitivity to abatacept or any of its components\n2. Has received any lymphocyte depleting agents including anti-CD20 monoclonal antibodies, alemtuzumab, ATG in the preceding 6 months\n3. Has received abatacept, cyclophosphamide, tumor necrosis factor inhibitors, or pulse steroids (defined as \\>15mg\u002Fkg\u002Fday of methylprednisone or corticosteroid equivalent) within the past 3 months\n4. Have started or increased any of the following immune modulating drugs within 3 months of enrolling and 3 months from initial CT chest: azathioprine, cyclosporine, tacrolimus, mercaptopurine, methotrexate, mycophenolate mofetil, or sirolimus\n5. History of HIV infection (positive PCR)\n6. Chronic untreated hepatitis B or C (positive PCR)\n7. Active tuberculosis (TB) by positive QuantiFERON gold. If history of latent TB, then must supply evidence of completing treatment.\n8. Persistent Epstein-Barr Virus (EBV) load ≥ 1,000 units\u002FmL blood checked twice at least 1 month apart\n9. Other uncontrolled infections\n10. Live vaccine given within 6 weeks of the start of the trial\n11. Malignancy or treated for malignancy within the past year\n12. Currently pregnant or breast feeding\n13. Life expectancy less than 1 month\n14. Subjects unwilling to self-administer or have a parent\u002Fcaregiver self-administer subcutaneous injections at home\n15. Other conditions that the investigators feel contraindicate participation in the study\n\nInclusion criteria for Extended Treatment Plan:\n\n* Patients must have completed the abatacept for the treatment of Interstitial Lung Disease in Common Variable Immunodeficiency (ABCVILD) trial\n* Patients must have demonstrated positive response to abatacept.\n* Patients must provide informed consent to participate in the Extended Treatment Plan.\n\nExclusion criteria for Extended Treatment Plan:\n\n• Patients who experienced SAEs during the original trial, and such SAEs were determined as related to treatment, or patients who in the opinion of the investigator would not benefit from the extended treatment option.",{"count":173,"type":21},38,[59],"There is no standard of care therapy for patients with granulomatous-lymphocytic interstitial lung disease (GLILD) seen in common variable immunodeficiency (CVID). Abatacept has recently looked promising for the treatment of patients with complex CVID. This study is a multi-site, phase II, randomized, blinded\u002Fplacebo-controlled clinical trial in pediatric and adult subjects to determine the efficacy of abatacept compared to placebo for treatment of subjects with GLILD in the context of CVID.\n\nFunding Source - FDA OOPD",[177,28],"Interstitial Lung Disease","2025-11-05",{"date":180,"type":40},"2025-11-06",{"date":182,"type":40},"2021-07-14",{"date":184,"type":21},"2026-07",{"name":186,"class":136},"Children's Hospital Medical Center, Cincinnati",6]