Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with Common Variable Immunodeficiency

Publication date: Available online 14 April 2017
Source:Current Problems in Diagnostic Radiology
Author(s): Jehan L. Shah, Sagar Amin, Nupur Verma, Tan-Lucien Mohammed
Common variable immunodeficiency (CVID) 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 female with CVID and GL-ILD and review the literature to summarize the most common radiological findings in order to raise the suspicion for GL-ILD on HRCT 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.IntroductionCommon variable immunodeficiency (CVID) is a primary immunodeficiency characterized by impaired immunoglobulin (Ig) production with low IgG and IgA levels resulting in the inability to make antibodies against infections.¹ While historically patients with CVID suffered most commonly from recurrent sinopulmonary infections, the use of IVIG and antibiotics have improved survival and shifted our focus to the increased risk for malignancy, mainly lymphoproliferative disorders, and autoimmune disorders, which are the main cause of mortality. Patients with CVID are often screened with HRCT to evaluate for active infections and the destructive changes associated with recurrent infections such as bronchiectasis in association with pulmonary function tests to gauge a patient′s respiratory function and tailor treatment options. One common and important distinction from pneumonia and bronchiectasis is granulomatous-lymphocytic interstitial lung disease (GL-ILD), which can be seen in 5–15% of patients with CVID and reduce the life span from an average of 29 years to 14 years. ² We present the case of a 25-year-old female diagnosed with CVID and biopsy proven GL-ILD. We will discuss the key pulmonary and extrapulmonary findings on HRCT, review the pathology, and differentiate GL-ILD from other common entities.



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