Ignite Creation Date:
2025-12-25 @ 12:43 AM
Ignite Modification Date:
2026-02-03 @ 10:43 AM
Study NCT ID:
NCT03648567
Status:
UNKNOWN
Last Update Posted:
2018-08-27
First Post:
2018-08-02
Is Gene Therapy:
True
Has Adverse Events:
False
Brief Title:
GLILD Diagnosed in Children and Young Adults With Common Variable Immunodeficiency
Sponsor:
Organization:
Raw JSON
{'hasResults': False, 'derivedSection': {'miscInfoModule': {'versionHolder': '2025-12-24'}, 'conditionBrowseModule': {'meshes': [{'id': 'D017074', 'term': 'Common Variable Immunodeficiency'}], 'ancestors': [{'id': 'D007153', 'term': 'Immunologic Deficiency Syndromes'}, {'id': 'D007154', 'term': 'Immune System Diseases'}]}}, 'protocolSection': {'designModule': {'studyType': 'OBSERVATIONAL', 'designInfo': {'timePerspective': 'RETROSPECTIVE', 'observationalModel': 'CASE_ONLY'}, 'enrollmentInfo': {'type': 'ESTIMATED', 'count': 24}, 'patientRegistry': False}, 'statusModule': {'overallStatus': 'UNKNOWN', 'lastKnownStatus': 'RECRUITING', 'startDateStruct': {'date': '2018-03-01', 'type': 'ACTUAL'}, 'expandedAccessInfo': {'hasExpandedAccess': False}, 'statusVerifiedDate': '2018-08', 'completionDateStruct': {'date': '2018-09-15', 'type': 'ESTIMATED'}, 'lastUpdateSubmitDate': '2018-08-23', 'studyFirstSubmitDate': '2018-08-02', 'studyFirstSubmitQcDate': '2018-08-23', 'lastUpdatePostDateStruct': {'date': '2018-08-27', 'type': 'ACTUAL'}, 'studyFirstPostDateStruct': {'date': '2018-08-27', 'type': 'ACTUAL'}, 'primaryCompletionDateStruct': {'date': '2018-09-01', 'type': 'ESTIMATED'}}, 'outcomesModule': {'primaryOutcomes': [{'measure': 'Lung biopsy', 'timeFrame': 'from 1998 to july 2018', 'description': 'Number of patients suspected of GLILD with lung biopsy whose characteristics corresponds to those defined by the British Lung Foundation'}], 'secondaryOutcomes': [{'measure': 'Clinical symptomatology', 'timeFrame': 'from 1998 to july 2018', 'description': 'Number of patients suspected of GLILD with significant clinical symptomatology'}, {'measure': 'Immunology', 'timeFrame': 'from 1998 to july 2018', 'description': 'Number of patients suspected of GLILD with a particular immunological profile'}, {'measure': 'Pulmonary function tests', 'timeFrame': 'from 1998 to july 2018', 'description': 'Number of patients suspected of GLILD with restrictive syndrome and/or carbon monoxide diffusion capacity alteration (Pulmonary Function Tests)'}, {'measure': 'CT chest in GLILD', 'timeFrame': 'from 1998 to july 2018', 'description': 'Number of patients suspected of GLILD with radiological characteristics corresponding to those defined by the British Lung foundation'}, {'measure': 'Broncho-alveolar lavage', 'timeFrame': 'from 1998 to july 2018', 'description': 'Number of patients suspected of GLILD with significant alteration of Broncho-alveolar Lavage'}, {'measure': 'GLILD Management', 'timeFrame': 'from 1998 to july 2018', 'description': 'Number of patients suspected of GLILD who received a treatment for this indication'}]}, 'oversightModule': {'oversightHasDmc': False, 'isFdaRegulatedDrug': False, 'isFdaRegulatedDevice': False}, 'conditionsModule': {'keywords': ['GLILD', 'Common variable immunodeficiency', 'children'], 'conditions': ['GLILD in a Population of Children and Young Adults']}, 'referencesModule': {'references': [{'pmid': '15316526', 'type': 'BACKGROUND', 'citation': 'Bates CA, Ellison MC, Lynch DA, Cool CD, Brown KK, Routes JM. Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency. J Allergy Clin Immunol. 2004 Aug;114(2):415-21. doi: 10.1016/j.jaci.2004.05.057.'}, {'pmid': '19900842', 'type': 'BACKGROUND', 'citation': 'Park JH, Levinson AI. Granulomatous-lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID). Clin Immunol. 2010 Feb;134(2):97-103. doi: 10.1016/j.clim.2009.10.002. Epub 2009 Nov 8.'}], 'seeAlsoLinks': [{'url': 'https://www.ncbi.nlm.nih.gov/pubmed/?term=British+Lung+Foundation%2FUnited+Kingdom+Primary+Immunodeficiency+Network+Consensus+Statement+on+the+Definition%2C+Diagnosis%2C+and+Management+of+Granulomatous-Lymphocytic+Interstitial+Lung+Disease+in+Common+Variable+Immunodeficiency+Disorders', 'label': 'British Lung Foundation'}]}, 'descriptionModule': {'briefSummary': '8 to 22% of patients with common variable immunodeficiency (CVID) will develop Granulomatous Lymphocytic Interstitial Lung Disease (GLILD), which has emerged as a major cause of mortality. Little is known about GLILD in children and young adults. The aim of this study was to describe the clinical, functional, radiological and pathological features of children and young adults diagnosed with GLILD.', 'detailedDescription': 'Variable common immunodeficiency (VCID) encompasses a heterogeneous group of primitive immunodeficiencies, with variable clinical and immunological settings, but globally characterized by hypogammaglobulinemia with significant reduction of Immunoglobulin G levels, often associated with a decrease in Immunoglobulin A and/or Immunoglobulin M levels, coupled with inability to produce antibodies in response to infection and/or immunization. VCID is the most common primary immunodeficiency, with an estimated prevalence between 1/10,000 and 1/50,000. With the introduction of high-dose, intravenous or subcutaneous immunoglobulins, number of infections, along with morbidity and induced mortality, has declined sharply in recent years. Conversely, non-infectious complications, such as autoimmune manifestations, inflammatory bowel diseases, enteropathies, hepatitis, lung disease and lymphoproliferation (up to lymphoma), increased considerably, reaching 70% of patients.\n\nGranulomatous Lymphocytic Interstitial Lung Disease is a non-infectious complication that can occur during the evolution of VCID and which is usually the pulmonary manifestation of a systemic polyclonal lymphoproliferative disease. GLILD contained both granulomatous and lymphoproliferative histopathologic patterns such as lymphocytic interstitial pneumonia , follicular bronchiolitis, and lymphoid hyperplasia. In recent series, approximately 8 to 22% of patients develop GLILD in VCID, and this complication is associated with increased mortality.\n\nAlthough there are now more studies conducted in the adult population, those in the pediatric population are only currently case report. To the best of our knowledge, very little data is available on this specific lung disease in the pediatric and young adults population.'}, 'eligibilityModule': {'sex': 'ALL', 'stdAges': ['CHILD', 'ADULT'], 'maximumAge': '25 Years', 'samplingMethod': 'NON_PROBABILITY_SAMPLE', 'studyPopulation': '* Children and young adults, aged from 0 to 25 years-old\n* with a diagnosis of Common Variable Immunodeficiency (marked decrease in IgG, at least less than -2 SD compared to the mean for age; associated with a decrease of at least one of the Immunoglobulin M or Immunoglobulin A isotypes, , absence of iso-haemagglutinins and/or poor vaccine response, with other defined causes of hypogammaglobulinemia excluded)\n* GLILD suspected according to the lung biopsy or CT chest', 'healthyVolunteers': False, 'eligibilityCriteria': 'Inclusion Criteria:\n\n* patient aged to 0 to 25 years old (at the diagnosis of GLILD)\n* diagnosed with a primary immunodeficiency syndrome "Common Variable Immunodeficiency" like, according to the 1999 American and European Societies for Immunodeficiency criteria\n* Suspected with GLILD (Granulomatous Lymphocytic Interstitial Lung Disease\n\nExclusion Criteria:\n\n* pulmonary diseases caused by other causes such as infectious or hypersensitivity pneumonitis'}, 'identificationModule': {'nctId': 'NCT03648567', 'acronym': 'pGLILD', 'briefTitle': 'GLILD Diagnosed in Children and Young Adults With Common Variable Immunodeficiency', 'organization': {'class': 'OTHER', 'fullName': 'Central Hospital, Nancy, France'}, 'officialTitle': 'Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) Diagnosed in Children and Young Adults With Common Variable Immunodeficiency', 'orgStudyIdInfo': {'id': 'PSS2017/p-GLILD-FOUYSSAC/NK'}}, 'contactsLocationsModule': {'locations': [{'zip': '25030', 'city': 'Besançon', 'status': 'RECRUITING', 'country': 'France', 'contacts': [{'name': 'Nathalie CHEIKH', 'role': 'CONTACT', 'email': 'ncheikh@chu-besancon.fr'}], 'facility': 'Chu Besancon', 'geoPoint': {'lat': 47.24878, 'lon': 6.01815}}, {'zip': '33000', 'city': 'Bordeaux', 'status': 'RECRUITING', 'country': 'France', 'contacts': [{'name': 'Nathalie Aladjidi', 'role': 'CONTACT', 'email': 'nathalie.aladjidi@chu-bordeaux.fr'}], 'facility': 'CHRU Bordeaux', 'geoPoint': {'lat': 44.84124, 'lon': -0.58046}}, {'zip': '21000', 'city': 'Dijon', 'status': 'RECRUITING', 'country': 'France', 'contacts': [{'name': 'Claire BRIANDET', 'role': 'CONTACT', 'email': 'claire.briandet@chu-dijon.fr'}], 'facility': 'Chru Dijon Bourgogne', 'geoPoint': {'lat': 47.31344, 'lon': 5.01391}}, {'zip': '34295', 'city': 'Montpellier', 'status': 'RECRUITING', 'country': 'France', 'contacts': [{'name': 'Eric JEZIORSKI', 'role': 'CONTACT', 'email': 'e-jeziorski@chu-montpellier.fr'}], 'facility': 'CHU Montpellier', 'geoPoint': {'lat': 43.61093, 'lon': 3.87635}}, {'zip': '54500', 'city': 'Nancy', 'status': 'RECRUITING', 'country': 'France', 'contacts': [{'name': 'Fanny FOUYSSAC', 'role': 'CONTACT', 'email': 'f.fouysac@chru-nancy.fr', 'phone': '003383154532'}, {'name': 'Mathilde Jouglet', 'role': 'CONTACT', 'email': 'mathilde.jouglet@hotmail.fr', 'phone': '003383154532'}], 'facility': 'CHRU Nancy', 'geoPoint': {'lat': 48.68439, 'lon': 6.18496}}, {'zip': '75015', 'city': 'Paris', 'status': 'RECRUITING', 'country': 'France', 'contacts': [{'name': 'Felipe SUAREZ', 'role': 'CONTACT', 'email': 'felipe.suarez@aphp.fr'}], 'facility': 'Hôpital Necker Enfants Malades', 'geoPoint': {'lat': 48.85341, 'lon': 2.3488}}], 'centralContacts': [{'name': 'Fanny FOUYSSAC', 'role': 'CONTACT', 'email': 'f.fouyssac@chru-nancy.fr', 'phone': '0033383154532'}, {'name': 'Mathilde JOUGLET', 'role': 'CONTACT', 'email': 'm.jouglet@chru-nancy.fr', 'phone': '0033383154532'}], 'overallOfficials': [{'name': 'Fanny FOUYSSAC', 'role': 'PRINCIPAL_INVESTIGATOR', 'affiliation': 'CHRU Nancy'}]}, 'ipdSharingStatementModule': {'ipdSharing': 'NO'}, 'sponsorCollaboratorsModule': {'leadSponsor': {'name': 'Central Hospital, Nancy, France', 'class': 'OTHER'}, 'responsibleParty': {'type': 'SPONSOR'}}}}