GRANULOMATOUS LYMPHOCYTIC INTERSTITIAL LUNG DISEASE: MOST COMMON NONINFECTIOUS PULMONARY COMPLICATION OF COMMON VARIABLE IMMUNODEFICIENCY

Abstract

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Common Variable Immunodeficiency (CVID) is the most common primary immunodeficiency. Patients with CVID suffer from recurrent sinopulmonary infections. Granulomatous lymphocytic interstitial lung disease (GLILD) is one of the dreaded noninfectious pulmonary complication of CVID. CASE PRESENTATION: A 28-year-old female was seen in Pulmonology clinic for evaluation of an abnormal CT scan of the chest, progressively worsening chest tightness, chronic productive cough and dyspnea on exertion for last 1 year. Patient had history of CVID, Chiari I malformation, and iron deficiency anemia. She worked with construction companies to monitor archeological changes. Vitals and physical exam on presentation were normal. Initial investigations showed thrombocytopenia; Sputum gram stain, fungal and acid-fast smear and aerobic and anaerobic cultures were unremarkable. CT scan of the chest, abdomen and pelvis with contrast showed diffuse reticulonodular pulmonary infiltrates predominantly in the basal lung fields, peri bronchial thickening in the lower lobes and mildly enlarged lymphadenopathy in the axilla, retroperitoneum and mesentery, and splenomegaly (13.5 cm). Differential diagnosis at the time were sarcoidosis, GLILD, fungal/atypical infections, vasculitis and malignancy. Bronchoscopy revealed acutely inflamed mucosa throughout the tracheobronchial tree, petechiae in right lower lobe. Bronchoalveolar lavage (BAL) in right medial segment and transbronchial biopsy (TBBx) in left lower lobe showed 54% lymphocytes, but no vasculitis, fungal/bacterial/viral infection, and malignancy. Working diagnosis was narrowed down to sarcoidosis and GLILD. Video assisted thoracoscopic surgery and open lung biopsy of right middle and lower lobes revealed nodular and diffuse mononuclear cell infiltrates and scattered non-necrotizing granulomas. 2nd opinion from a tertiary care center confirmed follicular bronchiolitis with associated non-necrotizing granulomatous inflammation and foci of fibrinous organizing pneumonia consistent with GLILD seen in CVID. DISCUSSION: GLLID is the most common cause of diffuse parenchymal lung disease in patients with CVID. It occurs in 8-22% of persons with CVID. The etiology of GLILD is unknown. It presents between the ages of 20 and 50. Presence of hypersplenism and polyarthritis increases the risk of GLILD in patients with CVID. Classic histopathologic pattern of GLILD is a cellular interstitial pneumonia with a combination of granulomatous and lymphoproliferative features. Optimal treatment is unknown. Glucocorticoids, IVIGs and immunosuppressive therapies have been tried. CONCLUSIONS: In patients with CVID development of respiratory signs or symptoms should be carefully evaluated for GLILD as presence of GLILD shortens the survival by approximately 50%. Reference #1: Fernández Perez ER. 2012 November. Granulomatous Lymphocytic Interstitial Lung Disease. Immunol Allergy Clin AM. 32(2012):621-32 Reference #2: Bates CA, Ellison MC, Lynch DA, et al. Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency. J Allergy Clin Immunol 2004;114(2):415–21. Reference #3: Tou w CM, van de Ven AA, de Jong PA, et al. Detection of pulmonary complications in common variable immunodeficiency. Pediatric Allergy Immunol 2010;21(5):793–805. DISCLOSURES: No relevant relationships by Shujaa Faryad, source=Web Response No relevant relationships by Rajamurugan Meenakshisundaram, source=Web Response