COMMON VARIABLE IMMUNODEFICIENCY-ASSOCIATED GRANULOMATOUS-LYMPHOCYTIC INTERSTITIAL LUNG DISEASE DIAGNOSED ON TRANSBRONCHIAL LUNG CRYOBIOPSY

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) is a rare entity involving granulomatous and lymphoproliferative inflammation of the small airways and pulmonary interstitium. Imaging reveals lower lung predominant ground glass and/or consolidative opacities and nodules. Histopathology may have features of lymphocytic interstitial pneumonia, follicular bronchiolitis and/or organizing pneumonia. GLILD is often seen as a manifestation of common variable immunodeficiency (CVID). Diagnosis requires lung biopsy as imaging findings are nonspecific. Surgical lung biopsy (SLB) is the gold standard in diagnosing interstitial lung disease. However, the relatively new, less invasive approach of transbronchial lung cryobiopsy (TBLC) has been increasingly considered for diagnostic purposes for diffuse parenchymal lung disease (DPLD). CASE PRESENTATION: A 58 year old male with a history of idiopathic thrombocytopenic purpura status-post splenectomy and rituximab presented with dyspnea on exertion, chest pressure and a chronic cough. CT chest revealed areas of consolidation and ground glass opacities in a lower lung distribution on a background of pulmonary fibrosis. Pulmonary function tests showed mild restriction and low diffusing capacity. He underwent outpatient TBLC with histopathologic findings of organizing pneumonia, patchy chronic inflammation, fibrosis and peribronchial lymphoid hyperplasia. Labs revealed low IgG and IgA, absence of member B cells, and deficiency of T cells. The patient was diagnosed with GLILD in setting of CVID. He was started on prednisone and IVIG with improvement in imaging findings and symptoms. DISCUSSION: GLILD is a granulomatous and lymphoproliferative disorder that occurs in 8-25% of patients with CVID. Patients may have shortness of breath and cough, as well as splenomegaly, cytopenia, and adenopathy. The constellation of signs and imaging findings are suggestive but not specific. Biopsy is needed for diagnosis. SLB is associated with relatively higher morbidity and mortality compared to TBLC, and necessitates hospitalization. TBLC has been proposed as a less invasive alternative to SLB. Advantages include lower mortality and complication rate, and no requirement for hospitalization. In this case, we demonstrate the utility of TBLC done as an outpatient in obtaining a diagnostic tissue sample, with clinical improvement on steroids and IVIG. There is no standard treatment for GLILD, but corticosteroids are recommended as first line therapy. Success has also been shown with azathioprine, ritxumab and IVIG. CONCLUSIONS: Transbronchial lung cryobiopsy is emerging as a less invasive alternative modality for obtaining tissue with less morbidity, less mortality and the advantage of being performed in the outpatient setting. Further studies regarding safety, diagnostic accuracy and yield in SLB versus TBLC are required to establish TBLC as common practice. Reference #1: Granulomatous-lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CIVD). Park JH, Levinson AI. Clin Immunol. 2010; 134(2):97. Reference #2: Transbronchial Lung Cryobiopsy in Idiopathic Pulmonary Fibrosis: A State of the Art Review. Adv Ther (2019) 36:2193-2204. Reference #3: British Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders. Hurst JR, Verma N. Lowe D, Daxendale HE, Jolles S, Kelleher P, Longhurst HJ, Patel Sy, Renzoni EA, Sander CR, Avery GR, Babar JL, Buckland MS, Burns S, Egner W, Gompels MM, Gordins P, Haddock JA, Hart SP, Hayman GR, Herriot R, Hoyles RK, Huissoon AP, Jacob J, Nicholson AG, Rassl DM, Sargur RB, Savic S, Seneviratne SL, Sheaff M, Vaailta PM, Walters GI, Whitehouse JL, Wright PA, Condliffe AM. J Allergy Clin Immunol Pract. 2017;5(4):938. Epub 2017 Mar 25. DISCLOSURES: No relevant relationships by Lawrence Benjamin, source=Web Response Speaker/Speaker's Bureau relationship with Boehringer-Ingelheim Pharmaceuticals Please note: $5001 - $20000 Added 04/07/2020 by Jaime Betancourt, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Vapotherm, Inc Please note: $5001 - $20000 Added 04/07/2020 by Jaime Betancourt, source=Web Response, value=Honoraria No relevant relationships by Houda Boucekkine, source=Web Response No relevant relationships by Scott Oh, source=Web Response