Granulomatous-lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID)

Abstract

Infectious complications of the lung occur quite frequently in patients with common variable immunodeficiency (CVID), a clinical syndrome that represents a primary immunodeficiency. However, there appears to be noninfectious pulmonary complications in association with CVID as well, and recently the term granulomatous-lymphocytic interstitial lung disease (GLILD) has been created to describe these noninfectious, diffuse lung disease complications that develop in CVID patients. They exhibit both granulomatous and lymphoproliferative histologic patterns, consisting of lymphocytic interstitial pneumonia (LIP), follicular bronchiolitis, and lymphoid hyperplasia. There are many unanswered questions surrounding this relatively unstudied entity. In an attempt to answer some of these questions, this review discusses in detail pathologic and clinical features of GLILD and its proposed pathogenesis with a particular attention to potential role of human herpesvirus 8 (HHV-8). Lastly, therapeutic approach is discussed to generate novel treatment strategy to better care for a subgroup of CVID patients afflicted with this entity.