Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II |

Tracheal Erosion in Dermatomyositis FREE TO VIEW

Eric Schilling, DO; Mark Rumbak, MD; Kimberly Cao, MD
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University of South Florida, Riverview, FL

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1095A. doi:10.1016/j.chest.2016.08.1202
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Dermatomyositis (DM) is known to result in a number of pulmonary complications. Our case evidences a novel condition in a patient with DM that resulted in a significantly erosive process in his trachea, with presentation and management not previously illustrated.

CASE PRESENTATION: A 60yo male with PMH of hepatitis C, HTN and DM2 presented with dysphagia. He had been diagnosed and treated for DM, after which he developed an aspiration pneumonia. Bronchoscopy revealed defects along the posterior tracheal wall, confirmed to be trachea-esophageal fistulae. Biopsies yielded necrotic tissue and were negative for malignancy. Subsequent bronchoscopies found significant progression with new lesions extending into the right mainstem and segmental bronchi. Fluoroscopy noted the fistulous tract extending into the right mediastinum. Steroids were unsuccessful in slowing progression, and necrotic debris was noted to drain into the right lung precipitating recurrent pneumonias and mediastinitis. As surgical intervention was no longer indicated, we pursued palliative placement of tracheal stents. Telescoping stents were placed from the left main-stem bronchus through the proximal trachea, bypassing these large tracheal lesions. This offered protection of the unaffected lung and a temporary therapeutic collapse in the poorly functioning right lung.

DISCUSSION: DM is a disease process known to cause numerous systemic symptoms. Myopathy is most common, but extra-neuromuscular symptoms can persist despite standard therapy. Rare cases of DM have resulted in panniculitis, vasculitis and malakoplakia, but rarely have mucocutaneous affects been described. Similarly, although ILD has been reported in up to 10% of DM cases, there have been no reports of large airway involvement. Our patient succumbed to tracheal erosions, likely as result of his autoimmunity. Airway stents have been a palliative tool to bipass obstructive lesions or bronchopleural fistulae. The use of abutting stent placement is helpful in treating larger lesions. Our case presented a particular challenge given the impressive area of erosion. We placed multiple stents from the left main bronchus to below the vocal cords. Our goal was to bypass the airway deficits to avoid any further pneumomediastinum. This effectively bypassed the right mainstem bronchus, allowing for atelectasis to promote healing of distal lesions. Results were intended to act as a “pneumonectomy” via endobronchial means in a patient that would not tolerate surgery. We believe this to be a novel approach to a complicated situation in which the trachea is compromised.

CONCLUSIONS: This case identifies a novel progression of a well-described illness, as well as an inventive way of managing a physiologic connundrum. Recognizing airway disease in an erosive process as illustrated here may lead to earlier intervention, even if only as a palliative means.

Reference #1: Le Goff B, Arthritis Rheum 2009; 61:108.

Reference #2: Dickey BF, Semin Arthritis Rheum 1984; 14:60.

DISCLOSURE: The following authors have nothing to disclose: Eric Schilling, Mark Rumbak, Kimberly Cao

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