Detail Article

Informations générales (source: ClinicalTrials.gov)

Numéro NCT

NCT03648567 Statut inconnu

Titre

Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) Diagnosed in Children and Young Adults With Common Variable Immunodeficiency (pGLILD)

Type

Observational

Pathologie

Déficits immunitaires
Déficit immunitaire commun variable

Phase

Promoteur

Central Hospital, Nancy, France (Voir sur ClinicalTrials)

Date de début

mars 2018

Date de fin

septembre 2018

Dernière mise à jour

29 juin 2024

Résumé

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.

Détail

Voir le détail 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. 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. Fermer le détail

Etablissements

Les établissements d'Île-de-France dont les données sont issues de ClinicalTrials.gov Origine et niveau de fiabilité des données
AP-HP - Hôpital Necker-Enfants Malades	Felipe SUAREZ	En recrutement IDF	Contact (sur clinicalTrials)
Les établissements hors Île-de-France dont les données sont issues de ClinicalTrials.gov Origine et niveau de fiabilité des données
CHRU Bordeaux - 33000 - Bordeaux - France	Nathalie Aladjidi	En recrutement	Contact (sur clinicalTrials)
Les établissements sans correspondance certaine dans le répertoire FINESS dont les données sont issues de ClinicalTrials.gov Origine et niveau de fiabilité des données
Chru Dijon Bourgogne - 21000 - Dijon - France	Claire BRIANDET	En recrutement	Contact (sur clinicalTrials)
CHRU Nancy - 54500 - Nancy - France	Fanny FOUYSSAC	En recrutement	Contact (sur clinicalTrials)
Chu Besancon - 25030 - Besançon - France	Nathalie CHEIKH	En recrutement	Contact (sur clinicalTrials)
CHU Montpellier - 34295 - Montpellier - France	Eric JEZIORSKI	En recrutement	Contact (sur clinicalTrials)

Critères

Genre

Tous

Nombre d'inclusion

Critère d'inclusion

Inclusion Criteria:

- patient aged to 0 to 25 years old (at the diagnosis of GLILD)

- 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

Critère de non inclusion

- pulmonary diseases caused by other causes such as infectious or hypersensitivity
pneumonitis

NCT03648567 - Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) Diagnosed in Children and Young Adults With Common Variable Immunodeficiency (pGLILD)

Informations générales
Etablissements
Critères
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