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 adults. Most patients suffer recurrent, mainly sinopulmonary, infections. Despite adequate IVIG replacement therapy, chronic lung disease continues to be a main cause morbidity mortality. The term granulomatous-lymphocytic interstitial (GLILD) frequently used describe associated with immune dysregulation primary deficiency, such as CVID. Aim: To case 10-year-old male CVID who developed GLILD his response treatment Rituximab. Discussion: Our patient young no genetic diagnosis, whose functions general condition continued deteriorate despite intravenous immunoglobulin therapy mycophenolate mofetil treatment. After diagnosis GLILD, we initiated 4-dose weekly course Rituximab prompt resolution disease. Although well described that accompanies manifestation dysregulation, it still under recognized, especially pediatric population. Among experts, there little uniformity when comes diagnostic approaches. Recent studies showed improved outcomes using combination Rituximab, our patient. Statement Novelty: We shed light on an important CVID, demonstrate excellent steroid sparing agent This crucial aspect considering therapeutic choices for