A case of Common Variable Immune Deficiency with Lung disease- Not Just Bronchiectasis

Introduction: Common Variable Immune Deficiency (CVID) is the most prevalent form of severe antibody deficiency in children and in adults. Most patients have recurrent infections, mainly sinopulmonary infections. Despite adequate IVIG replacement therapy chronic lung disease continues to be a main cause of morbidity and mortality. The term granulomatous-lymphocytic interstitial lung disease (GLILD) is frequently used to described the Interstitial lung disease associated with immune dysregulation in primary antibody deficiency such as CVID. Aim: To describe a 10-year-old boy with CVID who developed GLILD and his response to treatment with Rituximab. Discussion: Although GLILD is a well described condition that accompanies CVID as a manifestation of immune dysregulation, it is still under recognized, especially in the pediatric population. Among experts, there is little uniformity when it comes to diagnostic and treatment approaches. Recent studies showed improved outcomes when using combination therapy with Rituximab, such as in our case presentation. Statement of Novelty: This report discusses a case of CVID in a 10-year-old boy, with no genetic diagnosis, whose lung functions and general condition continued to deteriorate despite adequate IGRT and MMF treatment. After the diagnosis of GLILD we initiated treatment with a 4-dose weekly course of Rituximab with prompt resolution of his interstitial disease. In our case we shed light on GLILD, an important condition that accompanies CVID and demonstrate an excellent response to Rituximab-a steroid sparing agent, which is a crucial aspect when considering therapeutic choices for the pediatric population.