Common variable immunodeficiency-associated granulomatous and interstitial lung disease

Abstract

Common variable immunodeficiency (CVID) is the most common primary immunodeficiency characterized by a deficiency of immunoglobulins. Approximately 30% of the patients develop autoimmune and granulomatous disease. Similar to sarcoidosis, granulomatous disease in CVID can potentially affect all organs, but the lung is the most common. Interstitial lung disease (ILD) manifests in 5-15% of CVID patients, and is present already at the initial diagnosis in the majority of patients. The number of published studies addressing ILD in CVID is limited. However, recently, several studies added substantial knowledge to the field and are discussed within this review in the context of the literature. Histologically, ILD in CVID presents within the known patterns of sarcoid-like granuloma, organizing pneumonia, lymphocytic interstitial pneumonitis and nonspecific interstitial pneumonia. Often, these patterns are concomitantly found in the same patients. Three new articles were published which analyzed high-resolution computed tomography findings and response to treatment. In a considerable number of patients, ILD is stable over years and patients may not need any immunosuppressive treatment. Prednisone treatment is often used as the first-line treatment and studies suggest response to treatment in 50-66% of cases. In progressive disease with lung function impairment, combined immunosuppressive treatment is recommended.