Phase 2 Study of Abatacept for Treating Interstitial Lung Disease in Children and Adults With Common Variable Immunodeficiency

What this Trial Is About

Researchers are evaluating the use of abatacept for treating granulomatous-lymphocytic interstitial lung disease GLILD in patients with common variable immunodeficiency CVID, a condition without a standard therapy. This multi-site, phase II, randomized, blinded, placebo-controlled clinical trial involves both pediatric and adult participants. The study aims to determine how effective abatacept is compared to a placebo in managing GLILD associated with CVID. Participants are divided into two groups based on weight and age. Pediatric subjects weighing less than 50 kg receive open-label abatacept with dosing based on weight, while pediatric subjects weighing 50 kg or more and adults are randomized in a 12 ratio to receive either abatacept or a placebo weekly for six months. After six months, all participants receive weekly abatacept treatment. Following the initial 12 months, patients may continue abatacept for up to three years, with safety monitoring every three months. Throughout the study, participants undergo various assessments including high-resolution chest CT scans at six months to measure lung changes. Additional tests such as lung function tests, quality of life questionnaires, steroid usage, infection monitoring, and pediatric growth measurements are performed at six and twelve months. Safety is closely monitored through reports of adverse events and infections. The total duration of participation may extend up to three years depending on continued treatment.

Abatacept for the Treatment of Common Variable Immunodeficiency With Interstitial Lung Disease