GLILD Diagnosed in Children and Young Adults With Common Variable Immunodeficiency
NCT ID: NCT03648567
Last Updated: 2018-08-27
Study Results
The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.
Basic Information
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UNKNOWN
24 participants
OBSERVATIONAL
2018-03-01
2018-09-15
Brief Summary
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Detailed Description
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Granulomatous 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.
Although 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.
Conditions
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Study Design
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CASE_ONLY
RETROSPECTIVE
Eligibility Criteria
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Inclusion Criteria
* diagnosed with a primary immunodeficiency syndrome "Common Variable Immunodeficiency" like, according to the 1999 American and European Societies for Immunodeficiency criteria
* Suspected with GLILD (Granulomatous Lymphocytic Interstitial Lung Disease
Exclusion Criteria
25 Years
ALL
No
Sponsors
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Central Hospital, Nancy, France
OTHER
Responsible Party
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Principal Investigators
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Fanny FOUYSSAC
Role: PRINCIPAL_INVESTIGATOR
CHRU Nancy
Locations
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Chu Besancon
Besançon, , France
CHRU Bordeaux
Bordeaux, , France
Chru Dijon Bourgogne
Dijon, , France
CHU Montpellier
Montpellier, , France
CHRU Nancy
Nancy, , France
Hôpital Necker Enfants Malades
Paris, , France
Countries
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Central Contacts
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Facility Contacts
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References
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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.
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.
Related Links
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Other Identifiers
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PSS2017/p-GLILD-FOUYSSAC/NK
Identifier Type: -
Identifier Source: org_study_id
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