AN UNCOMMON CAUSE OF COUGH AND DYSPNEA DIAGNOSED BY BRONCHOSCOPY

Abstract

SESSION TITLE: Fellows Lung Pathology Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Tracheobronchial amyloidosis is a rare entity, characterized by symptoms of respiratory disease – cough, dyspnea, stridor, hemoptysis, hoarseness. Those with tracheobronchial involvement may have no systemic involvement. We present a very unusual case of tracheobronchial disease with cystic changes. CASE PRESENTATION: 58-year-old presented to pulmonary clinic with several months of cough and shortness of breath. Evaluation included a CT scan which showed thickened central airways with calcification and parenchymal lung cysts, along with normal pulmonary function test. Bronchoscopy revealed thickened mucosa and nodular appearance to trachea that occurs diffusely in the main trachea and bilateral right and left mainstem bronchi. Transbronchial biopsies were done and there was significant friability of tissue following biopsies. Gross pathology showed bronchial mucosa with lumpy amorphous eosinophilic deposits undergoing cartilaginous metaplasia and positive Congo red stain and apple-green birefringence in polarized light. Further characterization with laser-capture microdissection (LCM) with liquid chromatography-coupled tandem mass spectrometry (MS/MS) detected a peptide profile consistent with AL (lambda) – type amyloid deposition. Testing was negative for Sjogren’s. She underwent evaluation for systemic amyloidosis including bone marrow biopsy which showed normocellular bone marrow with morphologically normal trilineage hematopoiesis. DISCUSSION: Differential diagnosis of diffuse tracheal lesions includes tracheopathia osteoplastica, tracheobronchial amyloidosis, respiratory papillomatosis, infections, granulomatosis with polyangiitis, metastatic disease, and multifocal tracheal chondromata. Localized tracheobronchial amyloidosis is a relatively rare entity and patients should be screened for presence of systemic involvement. Currently there is no proven role for systemic chemotherapy in treatment and local radiation or debridement has been used to treat obstructive disease. Amyloidosis may also present as cystic changes near bronchocentric nodules or as nodular densities. CONCLUSIONS: A rare cause of diffuse tracheal lesions, tracheobronchial amyloidosis can present as cough and needs tissue for diagnosis. More research is needed to better elucidate treatment options for these patients Reference #1: Milani P, Basset M, Russo F, Foli A, Palladini G, Merini G. The Lung in Amyloidosis. Eur Respir Rev 2017; 26:170046 Reference #2: Capizzi SA, Betancourt E, Prakash UB. Tracheobronchial Amyloidosis. Mayo Clin Proc. 2000; 75:1148-1152 Reference #3: Neben-Wittich M, Foote RL, Kalra S. External Beam Radiation Therapy for Tracheobronchial Amyloidosis. Chest 2007: 132(1):262-267 DISCLOSURES: No relevant relationships by Yaoli Yang, source=Web Response