GLILD in a Population of Children and Young Adults
Conditions
Keywords
GLILD, Common variable immunodeficiency, children
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.
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.
Interventions
None listed
Sponsors
Central Hospital, Nancy, France
Study design
Observational model
CASE_ONLY
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
No minimum to 25 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Lung biopsy | 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
| Measure | Time frame | Description |
|---|---|---|
| Immunology | from 1998 to july 2018 | Number of patients suspected of GLILD with a particular immunological profile |
| Pulmonary function tests | from 1998 to july 2018 | Number of patients suspected of GLILD with restrictive syndrome and/or carbon monoxide diffusion capacity alteration (Pulmonary Function Tests) |
| Clinical symptomatology | from 1998 to july 2018 | Number of patients suspected of GLILD with significant clinical symptomatology |
| Broncho-alveolar lavage | from 1998 to july 2018 | Number of patients suspected of GLILD with significant alteration of Broncho-alveolar Lavage |
| GLILD Management | from 1998 to july 2018 | Number of patients suspected of GLILD who received a treatment for this indication |
| CT chest in GLILD | from 1998 to july 2018 | Number of patients suspected of GLILD with radiological characteristics corresponding to those defined by the British Lung foundation |
Countries
France
Contacts
Primary ContactFanny FOUYSSAC
Backup ContactMathilde JOUGLET
Outcome results
None listed