SESSION TITLE: Pulmonary Manifestations of Systemic Diesase SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: The incidence of recurrent pleural effusions is 1-2% in patients with systemic amyloidosis. Tracheobronchial amyloidosis (TBA) is a rare condition that primarily involves large airways. Patients usually present with dyspnea and wheezing. Pleural effusion is not a common manifestation of TBA. Herein, we present a rare case of primary TBA presenting as a recurrent unilateral pleural effusion. CASE PRESENTATON: An 81-year-old non-smoker female with a significant history of recent hip surgery, breast cancer in remission for 10 years, hypertension and coronary artery disease presented to the hospital with a one-day history of insidious onset of dyspnea and dry cough. She appeared to be in mild distress and had decreased air entry on the left chest along with expiratory wheezing on auscultation. The rest of her exam was unremarkable. Her chest x-ray showed a new large left-sided pleural effusion. She underwent thoracentesis; drained 1500 ml of yellow serous fluid. The pleural fluid analysis was predominantly lymphocytic, exudative, with negative cultures and cytology. A post-thoracentesis chest CT scan showed left lower lobe consolidation and a segmental collapse of the left upper lobe (LUL). On Bronchoscopy, an endobronchial lesion with near total obstruction of LUL bronchus was visualized. Subsequently, an endobronchial biopsy was performed. Pathology of the lesion was consistent with amyloid deposition confirmed by Congo Red staining. Further workup to rule out systemic amyloidosis was negative. The patient’s clinical, imaging, and pathology findings were consistent with primary light chain TBA with post-obstructive atelectasis and pleural effusion. The patient was not a candidate for Melphalan secondary to her poor functional status and she declined treatment with Rituximab. Accordingly, she underwent external beam radiation therapy and was started on methylprednisolone 40 mg intravenously for five days, followed by a two-week course of a tapered regimen of oral prednisone. Subsequent follow-up a month after discharge showed complete resolution of left-sided pleural effusion. DISCUSSION: Pleural effusion is common in systemic amyloidosis, especially in the setting of amyloid cardiomyopathy, and interstitial deposition of amyloid protein. The presence of pleural effusion in systemic amyloidosis is associated with an expected survival of 1.8 months. In the absence of systemic amyloidosis, it is unknown whether pleural effusion is a manifestation of isolated TBA. In TBA, almost half of chest X-rays are normal. A common finding includes lobar collapse, but not pleural effusion. To our knowledge, this is the first case of primary TBA associated pleural effusion which is responsive to both steroids and radiotherapy. CONCLUSIONS: In the absence systemic amyloidosis, amyloid cardiomyopathy, or pulmonary parenchymal amyloidosis, pleural effusion may also be a clinical manifestation of localized TBA. Reference #1: Tada, L., Anjum, H., Linville, W.K. and Surani, S. (2015). Recurrent pleural effusions occurring in association with primary pulmonary amyloidosis. Case reports in pulmonology, 2015. Reference #2: L Berk, John & O'Regan, Anthony & Skinner, Martha. (2002). Pulmonary and Tracheobronchial Amyloidosis. Seminars in respiratory and critical care medicine. 23. 155-65. 10.1055/s-2002-25304. DISCLOSURES: No relevant relationships by Thamer Sartawi, source=Web Response No relevant relationships by Mingchen Song, source=Web Response No relevant relationships by Rajagopal Sreedhar, source=Web Response