GLILD Diagnosed in Children and Young Adults With Common Variable Immunodeficiency

NCT ID: NCT03648567

Last Updated: 2018-08-27

Study Results

Results pending

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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Recruitment Status

UNKNOWN

Total Enrollment

24 participants

Study Classification

OBSERVATIONAL

Study Start Date

2018-03-01

Study Completion Date

2018-09-15

Brief Summary

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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.

Detailed Description

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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.

Conditions

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GLILD in a Population of Children and Young Adults

Study Design

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Observational Model Type

CASE_ONLY

Study Time Perspective

RETROSPECTIVE

Eligibility Criteria

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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

Exclusion Criteria

* pulmonary diseases caused by other causes such as infectious or hypersensitivity pneumonitis
Maximum Eligible Age

25 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Central Hospital, Nancy, France

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Fanny FOUYSSAC

Role: PRINCIPAL_INVESTIGATOR

CHRU Nancy

Locations

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Chu Besancon

Besançon, , France

Site Status RECRUITING

CHRU Bordeaux

Bordeaux, , France

Site Status RECRUITING

Chru Dijon Bourgogne

Dijon, , France

Site Status RECRUITING

CHU Montpellier

Montpellier, , France

Site Status RECRUITING

CHRU Nancy

Nancy, , France

Site Status RECRUITING

Hôpital Necker Enfants Malades

Paris, , France

Site Status RECRUITING

Countries

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France

Central Contacts

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Fanny FOUYSSAC

Role: CONTACT

0033383154532

Mathilde JOUGLET

Role: CONTACT

0033383154532

Facility Contacts

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Nathalie CHEIKH

Role: primary

Nathalie Aladjidi

Role: primary

Claire BRIANDET

Role: primary

Eric JEZIORSKI

Role: primary

Fanny FOUYSSAC

Role: primary

003383154532

Mathilde Jouglet

Role: backup

003383154532

Felipe SUAREZ

Role: primary

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.

Reference Type BACKGROUND
PMID: 15316526 (View on PubMed)

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.

Reference Type BACKGROUND
PMID: 19900842 (View on PubMed)

Other Identifiers

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PSS2017/p-GLILD-FOUYSSAC/NK

Identifier Type: -

Identifier Source: org_study_id

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