Short summary

Common Variable Immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency disorder among adults of whom 10-30% develop a sterile inflammatory process in the lungs called granulomatous and lymphocytic interstitial lung disease(GLILD). We will 1) increased knowledge on GLILD, 2) identify diagnostic tools (i.e. biomarkers for clinical use) for early identification and stratified CVID care, and 3) identify underlying disease mechanisms with special focus on therapy.

Rationale

The cause of disease is incompletely understood. Not only is GLILD assumed to be highly underdiagnosed, GLILD is a serious complication that is proposed to be associated with a poor prognosis and no consensus on management.

Description of the cohort

We will identify and register all CVID patients, 18 years or older, followed for care by infectious disease specialists in the Region of Southern and Central Denmark. We will screen all CVID patients for GLILD.

Data and biological material

RedCAP data Biobank with blood and fluid from bronkial alveolar lavage

Collaborating researchers and departments

South Danish Center for Interstitial Lung Diseases (SCILS), Department of Respiratory Medicine.

Department of Clinical Immunology, Department of Internal Medicine, Kolding Sygehus.

Department of Infectious Diseases, Aarhus University Hospital.

Department of Respiratory Medicine, Aarhus University Hospital