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Allergy and Airway |

A Case of Tracheobronchial Amyloidosis

Ryan Bohle, MD; Maria Picken, MD; Sunita Kumar, MD
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Loyola University Medical Center, Maywood, IL


Chest. 2013;144(4_MeetingAbstracts):60A. doi:10.1378/chest.1704930
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Abstract

SESSION TITLE: Bronchology Student/Resident Case Report Posters

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Amyloidosis isolated to the respiratory tract is an uncommon presentation1.

CASE PRESENTATION: A 69 year old Caucasian male initially presented to ENT with hoarseness. Thickening of nasal turbinates was biopsied revealing amyloid derived from kappa light chain, AL-kappa. He reported no other symptoms. He began treatment with steroids and initially with lenalinomide, which after adverse reaction was switched to bortezomib. One year diagnosis he developed dyspnea on exertion. After a syncopal event he was referred for pulmonary evaluation. On the day of his pulmonary appointment his vital signs were: 98 degrees F , BP 123/70, pulse 64, SpO2 98%. Pulmonary function tests showed normal spirometry, normal lung volumes, and near normal diffusion capacity. The echocardiogram showed an ejection fraction 40%. BNP was within normal limits at 43; EKG with normal sinus rhythm; no signs of conduction disease. However, chest CT demonstrated ground glass opacities in bilateral apices and diffuse thickening of the wall of trachea and major bronchi. Bronchoscopy revealed edematous, erythematous and friable airways which bled easily. Biopsy demonstrated amyloid in bronchiolar stroma. Given the progression of disease, the decision was made to continue steroids but add melphalan, an alkylating agent, combined with a newly approved proteosome inhibitor carfilzomib. His dyspnea almost completely resolved with 2 months of therapy. A follow up chest CT is pending. The abdominal fat pat biopsy by fine needle aspiration is negative for amyloid deposition, however a surgical fat biopsy, which would provide higher sensitivity, is pending.

DISCUSSION: According to a case series of 55 patients with pulmonary amyloidosis, published in the Annals of Internal Medicine2, only 17 patients had localized amyloidosis. Of the latter group, 4 cases had tracheobronchial amyloidosis. Usual presentation is with dyspnea and cough with possible hemoptysis in the 5th and 6th decades of life. Radiologic findings generally demonstrate thickened airways3. The mean survival of patients localized pulmonary amyloid has been reported to be less than 4 years1.

CONCLUSIONS: Unless a surgical fat pad biopsy shows evidence of systemic amyloidosis, this patient has amyloidosis limited to the respiratory tract.

Reference #1: Chu H, Zhao L, Zhang Z, Gui T, Yi X, Sun X. Clinical characteristics of amyloidosis with isolated respiratory system involvement: A review of 13 cases. Ann Thorac Med. 2012 Oct;7(4):243-9.

Reference #2: Utz JP, Swensen SJ, Gertz MA. Pulmonary amyloidosis. The Mayo Clinic experience from 1980 to 1993. Ann Intern Med. 1996;124:407-413.

Reference #3: Lachmann HJ, Hawkins PN. Amyloidosis and the lung. Chron Respir Dis. 2006;3:203-14.

DISCLOSURE: The following authors have nothing to disclose: Ryan Bohle, Maria Picken, Sunita Kumar

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