Granulomatous-Lymphocytic Interstitial Lung Disease in a Patient With Common Variable Immunodeficiency.

ABSTRACT

Common variable immunodeficiency is the most common primary immunodeficiency and consists of impaired immunoglobulin production causing recurrent sinopulmonary infections. The most common cause of mortality for this disorder, however, is from the development of malignancy and autoimmune disorders. One common entity that develops is a systemic granulomatous and lymphoproliferative disorder that can cause an interstitial lung disease more formally referred to as granulomatous-lymphocytic interstitial lung disease (GL-ILD). We discuss a case of a 25-year-old woman with common variable immunodeficiency and GL-ILD and review the literature to summarize the most common radiological findings to raise the suspicion for GL-ILD on high-resolution computed tomography and delineate this from infection and other mimickers. We will also review key histopathological characteristics for diagnosis and the clinical approach and treatment options for this rare disease.