A CASE OF ADULT-ONSET, STEROID-RESISTANT ASTHMA DIAGNOSED AS BRONCHOCENTRIC GRANULOMATOSIS RESPONSIVE TO MEPOLIZUMAB

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: Several asthma related diagnoses that involve granulomatous inflammation have been described in the literature. Bronchocentric granulomatosis (BG) is a histopathological finding characterized by the granulomatous necrotizing reaction centered in the airways. Asthmatic granulomatosis (AG) has features of autoimmunity, non-necrotizing granulomas, and physiologic features of asthma. CASE PRESENTATION: A 48-year-old man with adult onset asthma, GERD, allergic rhinitis, and atopic dermatitis, presented with chronic cough for 10 months. His cough began during a trip to Puerto Vallarta, Mexico, where he suffered from pneumonia requiring mechanical ventilation. Over the next year, he continued to suffer from recurrent pneumonia and chronic cough. He was treated with multiple courses of steroids without much relief of symptoms. He demonstrated persistently elevated CRP, serum eosinophils, and IgE levels. CT Chest showed pre-tracheal lymphadenopathy, ground glass opacities, and tree-in-bud nodules. Workup was negative for any fungal or rheumatologic serologies. Eventually, VATS wedge biopsy was performed and read as BG. The pathology was sent for and confirmed by a second opinion. Patient was started on mepolizumab and corticosteroids with full resolution of his cough. DISCUSSION: BG is an older entity first described in 1973 and was initially related to asthmatic patients with ABPA and to non-asthmatic patients with no identified etiologic agent. Current etiologies for non-asthmatic patients have been related to many diseases such as infectious etiologies (mycobacteria, pulmonary echinococcosis, Influenza A) and other diseases such as RA, CGD, and GPA. While his IgE and eosinophil count were elevated, our patient had neither IgE nor serum percipitins against Aspergillus or other mycoses ruling out ABPA. Our patient did demonstrate histopathologic findings of BG, however, was unresponsive to multiple courses of oral glucocorticoids. BG is rarely refractory to glucocorticoids, and there are no case reports demonstrating response to mepolizumab, which is used to treat severe eosinophilic asthma. This case shares features of both BG and AG in regards to its clinical presentation, histopathologic findings, and response to treatment. Our patient did not meet criteria for severe asthma and could not be appropriately categorized as BG related to asthma and ABPA. CONCLUSIONS: This case highlights overlap of BG, AG and other granulomatous lung diseases that comprise a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. We can conclude that our case is a unique presentation of idiopathic glucocorticoid-refractory-BG with shared features of AG, responsive to newer immunotherapy. As far as we can tell this is the first report of glucocorticoid-refractory BG responsive to mepolizumab. Reference #1: Liebow AA. Pulmonary angiitis and granulomatosis. Am Rev Respir Dis 1973;108:1-18. Reference #2: Wenzel SE, Vitari CA, Shende M, Strollo DC, Larkin A, Yousem SA. Am J Respir Crit Care Med. 2012 Sep 15;186(6):501-7. Reference #3: J.L. Myers, Bronchocentric granulomatosis. Disease or diagnosis? Chest 96 (1989) 3e4. DISCLOSURES: No relevant relationships by Bhavik Gupta, source=Web Response No relevant relationships by Brian Stephenson, source=Web Response No relevant relationships by Ajeet Vinayak, source=Web Response