CASE OF A GRANULOMATOUS-LYMPHOCYTIC INTERSTITIAL LUNG DISEASE IN A PATIENT WITH COMMON VARIABLE IMMUNODEFICIENCY DISORDER

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 1 SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/09/2018 03:45 PM - 04:45 PM INTRODUCTION: Common variable Immunodeficiency Disorder (CVID), is the most common primary immunodeficiency disorder. Approximately 30% of patients with CVID develop autoimmune and granulomatous disease. Interstitial lung disease (ILD) manifests in 5-15% of CVID patients and represents a diagnostic challenge. It may present with sarcoid like granulomas, organizing pneumonia, lymphocytic interstitial pneumonia and nonspecific interstitial pneumonia. We present one such case of granulomatous-lymphocytic interstitial lung disease (GL-ILD). CASE PRESENTATION: 60 year old African American non-smoking female with no significant underlying medical history presented with cough in March 2006. Family history consisted of mother with stomach cancer, aunt with bone cancer, and grandmother with liver cancer. On physical exam, she had palpable axillary and inguinal lymph nodes. She had an abnormal CT chest showing bilateral lung nodules along with mediastinal lymphadenopathy. CT abdomen/pelvis showed retroperitoneal and pelvic adenopathy. PET scan showed uptake in multiple regions in neck, chest, abdomen and pelvis. PPD and HIV tests were negative.Workup for connective tissue disease came back negative except for low IgA, low normal IgG, and reduced IgM levels. She had minimally elevated ACE level. Inguinal and left axillary lymph node biopsies were non-diagnostic, showing benign lymph nodes with reactive follicular hyperplasia. Flow cytometry was negative. Video-assisted thoracoscopic left lung biopsies showed nodular airway-centered lymphoid hyperplasia with associated scattered small non-necrotizing granulomas. Follicular bronchiolitis was present. Biopsies did not suggest sarcoidosis. Mycobacterial and fungal cultures were negative. She had serial CT chest, abdomen and pelvis for the next two years showing no progression of her disease. After 2008, she had slow progression of lymphadenopathy and was evaluated at a tertiary care center in 2012. Definitive diagnosis of GL-ILD was given at that point. She was placed on steroids for about a year with stabilization of her disease. Subsequently, she had worsening of her lymphadenopathy off steroids and has been maintained on IgG infusions. DISCUSSION: CVID is present in a considerable number of patients with a small subset presenting with cryptic ILD. Exhaustive diagnostic workup is usually needed including open lung biopsy. Patients with GL-ILD may remain stable over years after optimization of their immunoglobulin levels. Following this, steroids remain first line treatment and response may be variable. In progressive disease, immunosuppressive treatment is recommended. CONCLUSIONS: This case illustrates an example of a form of interstitial lung disease associated with CVID. When lung biopsies demonstrate lymphocytic infiltrate and/or granuloma in the lung, CVID should be considered in the differential. Reference #1: British Lung Foundation/United Kingdom PrimaryImmunodeficiency Network Consensus Statement. J Allergy Clin Immunol Pract. 2017 Jul - Aug;5(4):938-945. https://doi.org/10.1016/j.jaip.2017.01.021. Epub 2017 Mar 25. Reference #2: Granulomatous-lymphocytic interstitial lung disease as the first manifestation of common variable immunodeficiency. Clin Respir J. 2018 Jan;12(1):337-343. https://doi.org/10.1111/crj.12511. Epub 2016 Jun 22. DISCLOSURES: No relevant relationships by Riju Dasgupta, source=Web Response No relevant relationships by Asok Dasgupta, source=Web Response No relevant relationships by Chelsea Leipold, source=Web Response No relevant relationships by David Taylor, source=Web Response No relevant relationships by Paresh Timbadia, source=Web Response