SESSION TITLE: Pulmonary Pathology II SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/21/2019 3:15 PM - 4:15 PM INTRODUCTION: Diffuse Panbronchiolitis (DPB) is a rare condition most commonly seen in patients of Asian descent (1). We identified a Caucasian patient with alpha-1antitrypsin (α1AT) MS genotype, who was diagnosed with DPB after multi-disciplinary discussion (MDD). CASE PRESENTATION: A 41 y/o woman with a diagnosis of hypersensitivity pneumonitis (HP) was referred for environmental exposure evaluation by an occupational/environmental pulmonologist to find the cause of her HP. She had presented five years previously with cough productive of yellow sputum. She reported a history of seasonal allergies. Her cough did not improve with bronchodilator use. Prednisone did provide some relief at a dose of 40 mg daily but was discontinued due to insomnia and edema. Her chest CT showed tubular bronchiectasis in both lower lobes and peribronchial thickening with peripheral infiltrates. Surgical lung biopsy demonstrated features thought to be consistent with HP. Comprehensive environmental exposure history was unrevealing. Physical exam revealed intermittent coughing, diffuse crackles and expiratory low-pitched wheezing. Due to discordance between her clinical & exposure history, chest imaging and pathology, her case was presented at MDD conference. During this MDD, thoracic radiology impression was that her imaging represented an airways-centered process such as bronchiolitis. Thoracic pathology review found bronchiolocentric lymphocytic infiltrations with germinal centers distributed in the middle of the secondary lobule, interstitial lymphocytes, and foamy macrophages in the interstitium, sparing the alveolar spaces. The massive lymphocytic infiltration in a bronchiolocentric pattern along with interstitial foamy macrophages was consistent with the unique pathologic pattern of DPB. Additional laboratory tests found normal α1AT level (125), but abnormal genotype (MS). CFTR gene sequencing and autoimmune antibodies were normal. CBC showed no leukocytosis or eosinophilia. She was treated with erythromycin and noted improvement in cough. DISCUSSION: DPB can be a rare cause of cough in non-Asian patients. α1AT MS genotype has been associated with airway disease and may be the reason for this patient’s presentation (2). Here, MDD yielded a refinement in clinical diagnosis, which led to alteration in care. Treatment for DPB includes chronic macrolide use, a therapy different from traditional therapies for HP, and would have not been instituted without this diagnostic refinement. CONCLUSIONS: This case highlights the utility of MDD approach in increasing diagnostic confidence of rare pulmonary diseases. Reference #1: Poletti V, et al. Diffuse panbronchiolitis. Eur Respir J 2006; 28:862. Reference #2: Sandford AJ, et al. Z and S mutations of the α1-antitrypsin gene and the risk of chronic obstructive pulmonary disease. Am J Respir Cell Mol Biol 1999; 20: 287–291. DISCLOSURES: No relevant relationships by Cheryl Augenstein, source=Web Response grant recipient relationship with Department of Defense, Department of Veterans Affairs, CDC Please note: >$100000 Added 03/18/2019 by Stella Hines, source=Web Response, value=Grant/Research Support No relevant relationships by Mary Richert, source=Web Response No relevant relationships by Bethany Weiler-Lisowski, source=Web Response No relevant relationships by Sarah Williams, source=Web Response
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