Lung Pathology: Fellow Case Report Poster - Lung Pathology |

Polymyositis With Intersititial Lung Disease FREE TO VIEW

Yuriy Takhalov, MD; John Kileci, MD
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Rutgers, NJMS, West New York, NJ

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

Chest. 2016;150(4_S):781A. doi:10.1016/j.chest.2016.08.877
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SESSION TITLE: Fellow Case Report Poster - Lung Pathology

SESSION TYPE: Affiliate Case Report Poster

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

INTRODUCTION: Polymyositis (PM) and dermatomyositis (DM) are systemic inflammatory disorders that may be associated with diffuse interstitial lung disease (ILD). The frequency of ILD in PM-DM ranges between 5% and 30%1. We report case of a patient with no known past medical history presenting with Polymyositis and ILD

CASE PRESENTATION: 41 year old male with no past medical history presented from five months history of shortness of breath, dry cough, lower extremity edema with muscle weakness in all extremities. His social history was significant for current incarceration of 1 year, without tobacco, alcohol or illicit drug use. On presentation he was afebrile, normotensive, with an O2 saturation of 87% on room air. Physical exam was significant for bilateral crackles in both lungs, proximal muscle tenderness, and no skin lesions were noted. A CT scan of his chest that showed peripheral ground glass opacities and reticular increased densities suggestive of pulmonary fibrosis with an acute component. A left thigh biopsy showed inflammatory myopathy consistent with Polymyositis. Pulmonary function test done showed restrictive pattern with impaired diffusion capacity. He was started on Prednisone 1mg/kg/daily and his dyspnea started to improve on day three of treatment. Further lab work revealed elevated Anti-Jo-1 antibody and Aldolase enzyme levels

DISCUSSION: Lungs are the most common extra-muscular organ involved in PM/DM occurring in up to 30% of patients2. In one study surgical lung biopsies in 22 pts revealed that Non-specific interstitial pneumonia was the most common (82%) pattern seen in patients with PM and ILD. Given the rapid response to steroids, our suspicion is that our patient falls within the BOOP group. Overall survival rates by the same authors revealed that one, three, and five year survival was 86%, 74%, and 60% respectively. To date, 8 antisynthetase antibodies have been identified with Anti-Jo-1, anti-PL-7, and anti- PL-12 antibodies as the strongest markers for ILD in patients with PM/DM

CONCLUSIONS: In patients with both proximal muscle weakness and tenderness combined with acute respiratory distress, ILD from PM/DM should be on top of the differential. Treatment of ILD and underlying connective tissue disorder would be most beneficial in improving these patient's outcomes.

Reference #1: Douglas, et al. Polymyositis-Dermatomyositis-associated Interstitial Lung Disease Am J Respir Crit Care Med 2001; 164:1182-1185

DISCLOSURE: The following authors have nothing to disclose: Yuriy Takhalov, John Kileci

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