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Allergy and Airway |

Tracheobronchial Amyloidosis: Recognize Before Compromise

Erin Crowley, MD; Praveen Mathur, MBBS; W. Graham Carlos, MD
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Indiana University School of Medicine, Indianapolis, IN


Chest. 2013;144(4_MeetingAbstracts):50A. doi:10.1378/chest.1703294
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Abstract

SESSION TITLE: Airway 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: Localized pulmonary amyloidosis is classified into four types: tracheobronchial amyloidosis (TBA), nodular amyloidosis, infiltrating interstitial amyloidosis, and lymph node amyloidosis (1). We present a case of localized tracheobronchial pulmonary amyloidosis.

CASE PRESENTATION: A 60-year old man was referred for refractory bronchitis associated with abnormal chest imaging. His symptoms included a non-productive cough and shortness of breath that was worse upon awakening. He denied pleurisy, fevers, weight loss, and sick contacts. He also complained of inspiratory wheezing without identifiable triggers. A chest CT scan demonstrated “diffuse wall thickening in the right middle and upper lobe bronchi, with near occlusive narrowing (figure 1).” Further investigation included normal pulmonary function testing with methacholine challenge and a normal echocardiogram. Bronchoscopy with endobronchial biopsy was performed. The bronchoscopy demonstrated diffuse erythema of the mucosa throughout the tracheobronchial tree and prominent submucosal blood vessels (figure 2). Endobronchial biopsies returned congo red stain positive securing the diagnosis.

DISCUSSION: TBA is rare and given the nonspecific symptoms at presentation, diagnosis can be difficult. Coughing, wheezing, hemoptysis, exertional dyspnea, and hoarseness are some of the more common presenting complaints (1,2). In patients with TBA, bronchoscopy may show flat plaques of amyloid material or a nodule that has the appearance of a neoplasm. While these findings may be diffuse, multifocal, or localized, it is key for the diagnosis they not be associated with systemic amyloidosis (3). Given the small database of cases, there are no standardized means to follow progression or large studies on the most effective treatment. An array of therapeutic options exists, ranging from close observation to laser therapies. Currently there are no entirely successful therapies and the efficacy of various drugs is unclear (1). Low-dose radiation takes advantage of the radiosensitive nature of the amyloid protein producing plasma cells. Despite attempts at debulking and repeated laser treatments, the persistent nature and progressive airway narrowing of this disease typically leads to respiratory compromise.

CONCLUSIONS: TBA is a rare disease characterized by the development of amyloid proteins within the bronchial tree. Bronchoscopy is critical to both diagnosis and subsequent therapeutic procedures.

Reference #1: Haiqing Chu, Lan Zhao, et. al: Clinical characteristics of amyloidosis with isolated respiratory system involvement: A review of 13 cases. Annals of Thoracic Medicine. 2013; 7:243-49.

Reference #2: Capizzi S, Betancourt E, Prakash U: Tracheobronchial Amyloidosis. Mayo Clin Proc. 2000; 75:1148-152.

Reference #3: Utz J, Swensen S, Gertz M: Pulmonary Amloidosis The Mayo Clinic Experience from 1980 to 1993. Ann Intern Med. 1996; 124:407-413.

DISCLOSURE: The following authors have nothing to disclose: Erin Crowley, Praveen Mathur, W. Graham Carlos

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amyloidosis

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