COVID-19 is an emerging, rapidly evolving situation.
Get the latest public health information from CDC:

Get the latest research information from NIH: Menu

GLILD Diagnosed in Children and Young Adults With Common Variable Immunodeficiency (pGLILD)

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT03648567
Recruitment Status : Unknown
Verified August 2018 by Central Hospital, Nancy, France.
Recruitment status was:  Recruiting
First Posted : August 27, 2018
Last Update Posted : August 27, 2018
Information provided by (Responsible Party):
Central Hospital, Nancy, France

Brief Summary:
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.

Condition or disease
GLILD in a Population of Children and Young Adults

Detailed Description:

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.

Layout table for study information
Study Type : Observational
Estimated Enrollment : 24 participants
Observational Model: Case-Only
Time Perspective: Retrospective
Official Title: Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) Diagnosed in Children and Young Adults With Common Variable Immunodeficiency
Actual Study Start Date : March 1, 2018
Estimated Primary Completion Date : September 1, 2018
Estimated Study Completion Date : September 15, 2018

Primary Outcome Measures :
  1. Lung biopsy [ Time Frame: from 1998 to july 2018 ]
    Number of patients suspected of GLILD with lung biopsy whose characteristics corresponds to those defined by the British Lung Foundation

Secondary Outcome Measures :
  1. Clinical symptomatology [ Time Frame: from 1998 to july 2018 ]
    Number of patients suspected of GLILD with significant clinical symptomatology

  2. Immunology [ Time Frame: from 1998 to july 2018 ]
    Number of patients suspected of GLILD with a particular immunological profile

  3. Pulmonary function tests [ Time Frame: from 1998 to july 2018 ]
    Number of patients suspected of GLILD with restrictive syndrome and/or carbon monoxide diffusion capacity alteration (Pulmonary Function Tests)

  4. CT chest in GLILD [ Time Frame: from 1998 to july 2018 ]
    Number of patients suspected of GLILD with radiological characteristics corresponding to those defined by the British Lung foundation

  5. Broncho-alveolar lavage [ Time Frame: from 1998 to july 2018 ]
    Number of patients suspected of GLILD with significant alteration of Broncho-alveolar Lavage

  6. GLILD Management [ Time Frame: from 1998 to july 2018 ]
    Number of patients suspected of GLILD who received a treatment for this indication

Information from the National Library of Medicine

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.

Layout table for eligibility information
Ages Eligible for Study:   up to 25 Years   (Child, Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
  • Children and young adults, aged from 0 to 25 years-old
  • 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)
  • GLILD suspected according to the lung biopsy or CT chest

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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT03648567

Layout table for location contacts
Contact: Fanny FOUYSSAC 0033383154532
Contact: Mathilde JOUGLET 0033383154532

Layout table for location information
Chu Besancon Recruiting
Besançon, France, 25030
Contact: Nathalie CHEIKH   
CHRU Bordeaux Recruiting
Bordeaux, France, 33000
Contact: Nathalie Aladjidi   
Chru Dijon Bourgogne Recruiting
Dijon, France, 21000
Contact: Claire BRIANDET   
CHU Montpellier Recruiting
Montpellier, France, 34295
Contact: Eric JEZIORSKI   
CHRU Nancy Recruiting
Nancy, France, 54500
Contact: Fanny FOUYSSAC    003383154532   
Contact: Mathilde Jouglet    003383154532   
Hôpital Necker Enfants Malades Recruiting
Paris, France, 75015
Contact: Felipe SUAREZ   
Sponsors and Collaborators
Central Hospital, Nancy, France
Layout table for investigator information
Principal Investigator: Fanny FOUYSSAC CHRU Nancy
Additional Information:
Layout table for additonal information
Responsible Party: Central Hospital, Nancy, France Identifier: NCT03648567    
Other Study ID Numbers: PSS2017/p-GLILD-FOUYSSAC/NK
First Posted: August 27, 2018    Key Record Dates
Last Update Posted: August 27, 2018
Last Verified: August 2018
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

Layout table for additional information
Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Keywords provided by Central Hospital, Nancy, France:
Common variable immunodeficiency
Additional relevant MeSH terms:
Layout table for MeSH terms
Immunologic Deficiency Syndromes
Common Variable Immunodeficiency
Immune System Diseases