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Chung, Yoon, Choi, Song, Lee, and Yoo: A Case of Dermatomyositis Complicated with Pneumomediastinum Successfully treated with Cyclosporine A: a Case Report and Review of Literature
Case Report

J Rheum Dis 2014;21(6):317-321.

Published online: 31 October 2014

DOI: https://doi.org/10.4078/jrd.2014.21.6.317

A Case of Dermatomyositis Complicated with Pneumomediastinum Successfully treated with Cyclosporine A: a Case Report and Review of Literature

Joo-Young Chung, Ha Yong Yoon, Yun Jung Choi, Soo-Kyeong Song, Won Seok Lee, Wan-Hee Yoo

Division of Rheumatology, Department of Internal Medicine, Chonbuk National University Medical School and Research Institute of Clinical Medicine of Chonbuk National University Hospital-Chonbuk National University, Jeonju, Korea

Corresponding to: Wan-Hee Yoo, Division of Rheumatology, Department of Internal Medicine, Chonbuk National University Medical School and Research Institute of Clinical Medicine of Chonbuk National University Hospital-Chonbuk National University, San 2-20 Geumam-dong, Deokjin-gu, Jeonju 561-180, Korea. E-mail: ywhim@jbnu.ac.kr

Received 27 May 2013       Revised 17 January 2014       Accepted 23 January 2014

Copyright © 2014 The Korean College of Rheumatology

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Dermatomyositis (DM) is a systemic inflammatory disease affecting skeletal muscles and other organs. Spontaneous pneumomediastinum (PnM) has been previously reported as a rare complication of DM and it is known to occur more frequently in patients with interstitial lung disease (ILD). Here we report on a case of a 52-year-old woman with DM who developed spontaneous PnM, which was treated successfully with high-dose steroid pulse therapy and cyclosporine A (CsA). This case suggests that CsA can be an effective therapeutic agent in DM refractory to glucocorticoid therapy, with ILD or pulmonary fibrosis accompanied by DM. CsA should be considered as an initial immunosuppressive agent for patients with PnM in DM.

Keywords: Dermatomyositis, Pneumomediastinum, Interstitial lung disease, Cyclosporin

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Figure 1.
Periungual erythema (A) and Gottron's sign (B).
jrd-21-317f1.tif
Figure 2.
Spontaneous pneumomediastinum (A) with subcutaneous emphysema (C) and resolved state (B and D) after the treatment with high dose steroid and cyclosporine.
jrd-21-317f2.tif
Table 1.
Review of dermatomyositis with spontaneous pneumomediastinum
No References Year Sex/Age Anti-Jo1 Ab ILD Skin lesion Management after PnM Outcome
1 Bradley et al. (3) 1986 M/42 NA NA Yes MPD+AZA Resolution
2 Kobayashi et al. (16) 1989 49/F NA Yes Yes High dose PDS+mediastinal drainage Death
3 Matsuda et al. (16) 1993 41/F NA Yes (−) Pulse MPD+PDS+AZA Death
4 Santos et al. (16) 1995 41/F NA Yes (−) PDS+AZA Death
5 Santiago et al. (11) 1998 M/10 (−) Yes NA Pulse MPD+HCQ Death
6 JH Park et al. (9) 1999 56/F (−) Yes Yes O2 therapy Death
7 HY Lee et al. (17) 2000 36/F (−) Yes Yes MPD+HCQ+cyclophosphamide Death
8 Kono et al. (4) 2000 M/30 (−) Yes Yes Pulse MPD+CsA Resolution
9 Kono et al. (4) 2000 M/25 NA Yes Yes Pulse MPD+CsA+AZA Death
10 Kono et al. (4) 2000 M/23 NA Yes Yes Pulse MPD Resolution
11 Kono et al. (4) 2000 F/59 NA Yes (−) Pulse MPD Resolution
12 Nonomura et al. (8) 2001 M/39 NA Yes NA CsA Resolution
13 Kuroda et al. (8) 2003 M/46 (−) Yes No PDS+CsA Resolution
14 Bin Yoo et al. (14) 2006 F/40 (−) Yes Yes MPD+IVIG+CsA Resolution
15 Terao et al. (8) 2007 M/16 (−) Yes Yes PDS+CsA Resolution
16 HY Lee et al. (17) 2007 45/F (−) Yes Yes Pulse MPD+cyclophosphamide Death
17 Powell et al. (11) 2007 M/34 (−) Yes NA Pulse MPD+IVIG Death
18 Park et al. (11) 2009 F/35 (−) Yes Yes PDS+AZA+cyclophosphamide Resolution
19 Yoo et al. (8) 2009 F/38 (−) Yes Yes MPD+CsA Resolution
20 Masrouha et al. (12) 2009 M/66 NA Yes NA Pulse MPD Resolution
21 Miyazaki et al. (11) 2011 M/29 (−) Yes NA Pulse MPD+CsA Resolution
22 Miyazaki et al. (11) 2011 F/41 (−) Yes NA Pulse MPD+CsA+cyclophosphamide+IVIG Resolution
23 Miyazaki et al. (11) 2011 F/43 (−) Yes NA Pulse MPD+CsA+cyclophosphamide Resolution
24 Hutchinson et al. (13) 2010 F/25 (−) Yes Yes Rituximab+MPD+cyclophosphamide Resolution
25 Kim et al. (15) 2010 F/59 (−) Yes (−) Pulse MPD+CsA Resolution
26 Kim et al. (15) 2010 F/51 (−) Yes (−) Pulse MPD+CsA Resolution
27 Kim et al. (15) 2010 F/42 (−) Yes (−) Pulse MPD+CsA Resolution
28 Kim et al. (15) 2010 F/43 (−) Yes (−) PDS+HCQ+IVIG+CsA Resolution
29 Kim et al. (15) 2010 F/44 (−) Yes (−) PDS+CsA Resolution
30 Kim et al. (15) 2010 F/54 (−) Yes (−) PDS+IVIG+Tacrolimus Resolution
31 Present case 2013 F/52 (−) Yes Yes Pulse MPD+CsA Resolution

AZA: azathiorpine, CsA: cyclosporin A, HCQ: hydroxychloroquine, IVIG: intravenous immunoglobulin, MPD: methylprednisone, PDS: prednisolone.

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