LAUSR

Common variable immunodeficiency (CVID) disorders refer to a relatively common primary immune deficiency group of diseases that present with infectious and inflammatory complications secondary to defects in antibody production and sometimes in cellular immunity. The disorder often presents in middle age or later with recurrent sinopulmonary infections, bronchiectasis or a plethora of noninfectious complications such as autoimmune disorders, granulomatous interstitial lung disease (GLILD), gastrointestinal diseases, malignancies (including lymphoma), and multisystem granulomatous disease resembling sarcoidosis. Infusion of immunoglobulin by intravenous (IVIG) or subcutaneous (SCIG) is the mainstay of therapy. Management of complications is often difficult as immune suppression may be necessary in these conditions, and entails the use of medications and biologicals which may further increase the risk for infections. Specifically, bronchiectasis, granulomatous lymphocytic interstitial lung disease, repeated sinopulmonary infections and malignancies are sequelae of antibody deficiency that may present to the pulmonologist. This review will provide an updated understanding of the molecular aspects, differential diagnosis, presentations and the management of CVID disorders.