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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. Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details.
 
ClinicalTrials.gov Identifier: NCT03648567
Recruitment Status : Recruiting
First Posted : August 27, 2018
Last Update Posted : August 27, 2018
Sponsor:
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.

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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



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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
Criteria

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 ClinicalTrials.gov identifier (NCT number): NCT03648567


Contacts
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Contact: Fanny FOUYSSAC 0033383154532 f.fouyssac@chru-nancy.fr
Contact: Mathilde JOUGLET 0033383154532 m.jouglet@chru-nancy.fr

Locations
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France
Chu Besancon Recruiting
Besançon, France, 25030
Contact: Nathalie CHEIKH       ncheikh@chu-besancon.fr   
CHRU Bordeaux Recruiting
Bordeaux, France, 33000
Contact: Nathalie Aladjidi       nathalie.aladjidi@chu-bordeaux.fr   
Chru Dijon Bourgogne Recruiting
Dijon, France, 21000
Contact: Claire BRIANDET       claire.briandet@chu-dijon.fr   
CHU Montpellier Recruiting
Montpellier, France, 34295
Contact: Eric JEZIORSKI       e-jeziorski@chu-montpellier.fr   
CHRU Nancy Recruiting
Nancy, France, 54500
Contact: Fanny FOUYSSAC    003383154532    f.fouysac@chru-nancy.fr   
Contact: Mathilde Jouglet    003383154532    mathilde.jouglet@hotmail.fr   
Hôpital Necker Enfants Malades Recruiting
Paris, France, 75015
Contact: Felipe SUAREZ       felipe.suarez@aphp.fr   
Sponsors and Collaborators
Central Hospital, Nancy, France
Investigators
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Principal Investigator: Fanny FOUYSSAC CHRU Nancy

Additional Information:
Publications:
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Responsible Party: Central Hospital, Nancy, France
ClinicalTrials.gov 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

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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:
GLILD
Common variable immunodeficiency
children
Additional relevant MeSH terms:
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Immunologic Deficiency Syndromes
Common Variable Immunodeficiency
Immune System Diseases