GLILD Diagnosed in Children and Young Adults With Common Variable Immunodeficiency

GLILD in a Population of Children and Young Adults Clinical Trial

— pGLILD  

Official title:

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT03648567
• Other study ID #: PSS2017/p-GLILD-FOUYSSAC/NK
• Status: Recruiting
• Start date: March 1, 2018
• Est. completion date: September 15, 2018

Study information

• Verified date: August 2018
• Source: Central Hospital, Nancy, France
• Contact: Fanny FOUYSSAC
• Phone: 0033383154532
• Email: f.fouyssac[@]chru-nancy.fr
• Is FDA regulated: No
• Study type: Observational

Clinical 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. The aim of this study was to describe the clinical, functional, radiological and pathological features of children and young adults diagnosed with GLILD.

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 24
• Est. completion date: September 15, 2018
• Est. primary completion date: September 1, 2018
• Accepts healthy volunteers: No
• Gender: All
• Age group: N/A to 25 Years

Eligibility

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

Related Conditions & MeSH terms

• Common Variable Immunodeficiency
• GLILD in a Population of Children and Young Adults
• Immunologic Deficiency Syndromes

Locations

Country	Name	City	State
France	Chu Besancon	Besançon
France	CHRU Bordeaux	Bordeaux
France	Chru Dijon Bourgogne	Dijon
France	CHU Montpellier	Montpellier
France	CHRU Nancy	Nancy
France	Hôpital Necker Enfants Malades	Paris

Sponsors (1)

Lead Sponsor	Collaborator
Central Hospital, Nancy, France

Country where clinical trial is conducted

France, 

References & Publications (2)

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. — View Citation

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. Review. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Lung biopsy	Number of patients suspected of GLILD with lung biopsy whose characteristics corresponds to those defined by the British Lung Foundation	from 1998 to july 2018
Secondary	Clinical symptomatology	Number of patients suspected of GLILD with significant clinical symptomatology	from 1998 to july 2018
Secondary	Immunology	Number of patients suspected of GLILD with a particular immunological profile	from 1998 to july 2018
Secondary	Pulmonary function tests	Number of patients suspected of GLILD with restrictive syndrome and/or carbon monoxide diffusion capacity alteration (Pulmonary Function Tests)	from 1998 to july 2018
Secondary	CT chest in GLILD	Number of patients suspected of GLILD with radiological characteristics corresponding to those defined by the British Lung foundation	from 1998 to july 2018
Secondary	Broncho-alveolar lavage	Number of patients suspected of GLILD with significant alteration of Broncho-alveolar Lavage	from 1998 to july 2018
Secondary	GLILD Management	Number of patients suspected of GLILD who received a treatment for this indication	from 1998 to july 2018

External Links

•   British Lung Foundation