GLILD in a Population of Children and Young AdultspGLILDNCT03648567
GLILD Diagnosed in Children and Young Adults With Common Variable Immunodeficiency
An observational study of GLILD in a Population of Children and Young Adults, run by Central Hospital, Nancy, France at 6 sites in France.
Trial 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. Read more about this study
- Who is it for?
- Ages Up to 25 years
- Conditions the study is for
- GLILD in a Population of Children and Young Adults
- How many people
- 24 participants
- Where
- 6 sitesFrance
- Study dates
- 1 Mar 2018to 15 Sep 2018
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages Up to 25 years
- All sexes
- No healthy volunteers
- Child, Adult
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
Who may be able to join
- 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
Who cannot join
- pulmonary diseases caused by other causes such as infectious or hypersensitivity pneumonitis
Trial locations and contacts
6 sites in 1 country.
6 sites in 1 country
France6 sites, 6 recruiting
- Recruiting
Chu Besancon
Besançon, 25030
Nathalie CHEIKH, ncheikh@chu-besancon.fr
- Recruiting
CHRU Bordeaux
Bordeaux, 33000
Nathalie Aladjidi, nathalie.aladjidi@chu-bordeaux.fr
- Recruiting
Chru Dijon Bourgogne
Dijon, 21000
Claire BRIANDET, claire.briandet@chu-dijon.fr
- Recruiting
CHU Montpellier
Montpellier, 34295
Eric JEZIORSKI, e-jeziorski@chu-montpellier.fr
- Recruiting
CHRU Nancy
Nancy, 54500
Fanny FOUYSSAC, 003383154532, f.fouysac@chru-nancy.fr
Mathilde Jouglet, 003383154532, mathilde.jouglet@hotmail.fr
- Recruiting
Hôpital Necker Enfants Malades
Paris, 75015
Felipe SUAREZ, felipe.suarez@aphp.fr
About this study
Official title: Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) Diagnosed in Children and Young Adults With Common Variable Immunodeficiency
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.
Read the 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.
- Keywords
- GLILD, Common variable immunodeficiency, children
- Medical subject headings
- Common Variable Immunodeficiency
What is being measured
The primary outcome is the main question the study is designed to answer.
Lung biopsy
Number of patients suspected of GLILD with lung biopsy whose characteristics corresponds to those defined by the British Lung Foundation
Measured: from 1998 to july 2018
Secondary outcomes (6)
Clinical symptomatology
Number of patients suspected of GLILD with significant clinical symptomatology
Measured: from 1998 to july 2018
Immunology
Number of patients suspected of GLILD with a particular immunological profile
Measured: from 1998 to july 2018
Pulmonary function tests
Number of patients suspected of GLILD with restrictive syndrome and/or carbon monoxide diffusion capacity alteration (Pulmonary Function Tests)
Measured: from 1998 to july 2018
CT chest in GLILD
Number of patients suspected of GLILD with radiological characteristics corresponding to those defined by the British Lung foundation
Measured: from 1998 to july 2018
Broncho-alveolar lavage
Number of patients suspected of GLILD with significant alteration of Broncho-alveolar Lavage
Measured: from 1998 to july 2018
GLILD Management
Number of patients suspected of GLILD who received a treatment for this indication
Measured: from 1998 to july 2018
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted27 Aug 2018
- Study start1 Mar 2018
- Primary completion1 Sep 2018 (estimated)
- Study completion15 Sep 2018 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (2)Papers the sponsor has linked to this study.
- Other trials for GLILD in a Population of Children and Young AdultsStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Case only
- Time perspective
- Retrospective
- Enrolment
- 24 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Central Hospital, Nancy, France
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Fanny FOUYSSAC, Principal investigator, CHRU Nancy
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT03648567
- Sponsor's protocol ID
- PSS2017/p-GLILD-FOUYSSAC/NK
- Acronym
- pGLILD
- Status last verified
- Aug 2018
- First submitted
- 2 Aug 2018
- Last updated
- 27 Aug 2018
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
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.
PubMed 15316526Park 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.
PubMed 19900842
Study at a glance
From the registry record, last updated by the sponsor on 27 Aug 2018. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) Diagnosed in Children and Young Adults With Common Variable Immunodeficiency
- ClinicalTrials.gov ID
- NCT03648567
- Acronym / sponsor ID
- pGLILD / PSS2017/p-GLILD-FOUYSSAC/NK
- Keywords
- GLILD, Common variable immunodeficiency, children
- Study type
- Observational
- Recruitment status
- Unknown status
- Enrolment
- 24 participants (estimated)
- Lead sponsor
- Central Hospital, Nancy, FranceUniversity, hospital or other
- Study start
- 1 Mar 2018 (actual)
- Primary completion
- 1 Sep 2018 (estimated)
- Study completion
- 15 Sep 2018 (estimated)
- First posted
- 27 Aug 2018 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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
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.
Study design
- Observational model
- Case only
- Time perspective
- Retrospective
Who may qualify
- Ages
- Up to 25 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child, Adult
- 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
Key inclusion criteria (3 listed)
- 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
Key exclusion criteria (1 listed)
- pulmonary diseases caused by other causes such as infectious or hypersensitivity pneumonitis
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
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
6 secondary outcomes
Clinical symptomatology
Time frame: from 1998 to july 2018
Immunology
Time frame: from 1998 to july 2018
Pulmonary function tests
Time frame: from 1998 to july 2018
CT chest in GLILD
Time frame: from 1998 to july 2018
Broncho-alveolar lavage
Time frame: from 1998 to july 2018
GLILD Management
Time frame: from 1998 to july 2018
Results and safety
Results are not available in the source record.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 27 Aug 2018.
- Background: PubMed 15316526Bates 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.
- Background: PubMed 19900842Park 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.
- British Lung FoundationLink provided by the sponsor.
- Individual participant dataSharing planned: No
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Fanny FOUYSSAC principal investigator
CHRU Nancy
6 sites in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of GLILD in a Population of Children and Young Adults, run by Central Hospital, Nancy, France at 6 sites in France.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 3 inclusion and 1 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: status not confirmed, as of the sponsor's last update on 27 Aug 2018. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 6 sites in France. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
Results are not available in the source record.
How can a doctor refer a patient or contact the research team?
Contact Fanny FOUYSSAC (0033383154532, f.fouyssac@chru-nancy.fr), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Lung biopsy (time frame: from 1998 to july 2018). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT03648567, which also lists 2 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 27 Aug 2018.