J Korean Rheum Assoc.  2010 Jun;17(2):143-152. 10.4078/jkra.2010.17.2.143.

Clinicial Significance of Spontaneous Pneumomediastinum in Dermatomyositis/Polymyositis

• Kim JJ
• Kim D
• Kim EK
• Sohn IW
• Jung KH
• Choi CB
• Sung YK
• Jun JB
• Uhm WS
• Kim TH
• Bae SC
• Yoo DH

• KMID: 2201957
• DOI: http://doi.org/10.4078/jkra.2010.17.2.143

Abstract

OBJECTIVE
Pneumomediastinum (PnM), a rare complication of dermatomyositis and polymyositis (DM/PM), is sporadic and has an unclear pathogenesis. PnM is almost always associated with interstitial lung disease (ILD), and is a poor prognostic factor in inflammatory myositis patients. We studied the prevalence of PnM in Korean DM/PM and its clinical significance.
METHODS
We retrospectively studied the medical records of 161 patients diagnosed with DM/PM meeting Bohan-Peter's criteria at Hanyang University Hospital for Rheumatic Diseases from 1995 to 2010. We collected following findings; demographic data, diagnosis, lung involvement, cause of death, and duration from diagnosis to death.
RESULTS
One hundred nineteen patients (73.9%) were DM and 42 patients (26.1%) were PM. Eighty three patients (51.6%) developed ILD at diagnosis or during follow up. Eighteen patients (11.2%) died because of ILD aggravation, infection, or malignancy. The mean duration from diagnosis to death was 11.5 months, with 10 patients (6.2%) dying from from ILD aggravation but none with spontaneous PnM. 6 patients (3.7%) presented with PnM, and it was associated with ILD worsening in all cases. PnM resolved with O2 inhalation, corticosteroids, and/or immunosuppressive agents after 11 weeks (mean) of therapy.
CONCLUSION
PnM is rare but associates with DM and aggravation of ILD. PnM does not usually cause fatalities and can be cured by appropriate therapy.

Keyword

Pneumomediastinum; Dermatomyositis/polymyositis; Interstitial lung disease; Prognosis

MeSH Terms

Adrenal Cortex Hormones
Cause of Death
Dermatomyositis
Follow-Up Studies
Humans
Immunosuppressive Agents
Inhalation
Lung
Lung Diseases, Interstitial
Mediastinal Emphysema
Medical Records
Myositis
Polymyositis
Prevalence
Prognosis
Retrospective Studies
Rheumatic Diseases
Adrenal Cortex Hormones
Immunosuppressive Agents

Reference

1). Dickey BF., Myers AR. Pulmonary disease in polymyositis/dermatomyositis. Semin Arthritis Rheum. 1984. 14:60–76.

2). Marie I., Hachulla E., Cherin P., Dominique S., Hatron PY., Hellot MF, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002. 47:614–22.

3). Kang EH., Lee EB., Shin KC., Im CH., Chung DH., Han SK, et al. Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology (Oxford). 2005. 44:1282–6.

4). Kim DH., Park JH., Chei CS., Hwang SW., Kim HY., Yoo BH. Spontaneous Pneumomediastinum: clinical investigation. Korean J Thorac Cardiovasc Surg. 2006. 39:220–5.

5). Lee SY., Jo YM., Kim MS., Kim HI., Lee SW., Chung WT. A Case of Pneumomediastinum in the Course of Treatment in Lupus Myocarditis. J Korean Rheum Assoc. 2008. 15:322–7.

6). Patel A., Kesler B., Wise RA. Persistent pneumomediastinum in interstitial fibrosis associated with rheumatoid arthritis: treatment with high-concentration oxygen. Chest. 2000. 117:1809–13.

7). Kishi K., Kashiwabara K., Narushima K., Sarashina G., Kobayashi K., Yagyu H, et al. Mixed connective tissue disease-associated interstitial pneumonia complicated by pneumomediastinum during prednisolone therapy. Nihon Kokyuki Gakkai Zasshi. 2000. 38:480–4.

8). Matsuda Y., Tomii M., Kashiwazaki S. Fatal pneumomediastinum in dermatomyositis without creatine kinase elevation. Intern Med. 1993. 32:643–7.

9). Yamanishi Y., Maeda H., Konishi F., Hiyama K., Yamana S., Ishioka S, et al. Dermatomyositis associated with rapidly progressive fatal interstitial pneumonitis and pneumomediastinum. Scand J Rheumatol. 1999. 28:58–61.

10). Kuroda T., Morikawa H., Satou T., Tanabe Y., Murakami S., Ito S, et al. A case of dermatomyositis complicated with pneumomediastinum successfully treated with cyclosporin A. Clin Rheumatol. 2003. 22:45–8.

11). Rhee K., Jung C., Bae J., So M., Oh J., Kim S, et al. A case of dermatomyositis complicated with Pneumomediastinum successfully treated with Cyclosporin A. J Korean Rheum Assoc. 2006. 13:160–5.

12). Yoshida K., Kurosaka D., Kingetsu I., Hirai K., Yamada A. Pneumomediastinum in dermatomyositis itself is not a poor prognostic factor: report of a case and review of the literature. Rheumatol Int. 2008. 28:913–7.

13). Bohan A., Peter JB. Polymyositis and dermatomyositis (first of two parts) N Engl J Med. 1975. 292:344–7.

14). Lynch D., Travis W., Muller N., Galvin J., Hansell D., Grenier P, et al. Idiopathic Interstitial Pneumonias: CT Features. Radiology. 2005. 236:10.

15). Kim HI., Back HK., Jung JK., Jo YM., Lee SY., Lee SW, et al. Prognostic factors affecting survival rate in inflammatory myositis. J Korean Rheum Assoc. 2009. 6:108–14.

16). Schwarz MI., Matthay RA., Sahn SA., Stanford RE., Marmorstein BL., Scheinhorn DJ. Interstitial lung disease in polymyositis and dermatomyositis: analysis of six cases and review of the literature. Medicine (Baltimore). 1976. 55:89–104.

17). Kono H., Inokuma S., Nakayama H., Suzuki M. Pneumomediastinum in dermatomyositis: association with cutaneous vasculopathy. Ann Rheum Dis. 2000. 59:372–6.

18). Barvaux V., Van Mullem X., Pieters T., Houssiau F. Persistent pneumomediastinum and dermatomyositis: a case report and review of the literature. Clin Rheumatol. 2001. 20:359–61.

19). Terao M., Ozawa K., Inui S., Murota H., Yokomi A., Itami S. A case of dermatomyositis complicated with pneumomediastinum. Mod Rheumatol. 2007. 17:156–9.

20). de Souza Neves F., Shinjo S., Carvalho J., Levy-Neto M., Borges C. Spontaneous pneumomediastinum and dermatomyositis may be a not so rare association: report of a case and review of the literature. Clin Rheumatol. 2007. 26:105–7.

21). Jansen T., Barrera P., Van Engelen B., Cox N., Laan R., Van de Putte L. Dermatomyositis with subclinical myositis and spontaneous pneumomediastinum with pneumothorax: case report and review of the literature. Clin Exp Rheumatol. 1998. 16:733–5.

22). Cicuttini FM., Fraser KJ. Recurrent pneumomediastinum in adult dermatomyositis. J Rheumatol. 1989. 16:384–6.

23). Tong S., Shi X., Su J., Zhao Y., Zhang F. Clinical analysis of pneumomediastinum complicated in polymyositis and dermatomyositis. Zhonghua Yi Xue Za Zhi. 2006. 86:624.

24). Le Goff B., Cherin P., Cantagrel A., Gayraud M., Hachulla E., Laborde F, et al. Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Care Res. 2008. 61:108–18.

25). Park SH., Kum YS., Kim KC., Choe JY., Kim SK. Pneumomediastinum and subcutaneous emphysema secondary to amyopathic dermatomyositis with cryptogenic organizing pneumonia in invasive breast cancer: a case report and review of literature. Rheumatol Int. 2009. 29:1231–5.

26). Kim HJ., Hong YK., Yoo WH. Dermatomyositis, complicated with pneumomediastinum, successfully treated with cyclosporine A: a case report and review of literature. Rheumatol Int. 2009. 29:1101–4.

27). Oh JH., Ko JJ., Lee CK., Ko YH., Yun KS., Lim YS, et al. A case of spontaneous pneumomediastinum and subcutaneous emphysema in dermatomyositis: Including analysis of cases with dermatomyositis and pneumomediastinum in the literature. Koreran J Med. 1998. 55:131–6.

28). Park JH., Park SH., Kim PS., Lee JW., Yoo WH., Joo YS, et al. Fatal pneumomediastinum and subcutaneous emphysema in a patient with dermatomyositis. J Korean Rheum Assoc. 1999. 6:287.

29). Song JS., Park YB., Lee JG., Kwon KH., Lee WK., Suh CH, et al. Two cases of spontaneous pneumomediastinum in dermatomyositis. J Korean Rheum Assoc. 1998. 5:152–7.

30). Lee HY., Moon HI., Kang KY., Ju JH., Park SH., Kim HY. Two cases of Spontaneous pneumomediastinum in amyopathic dermatomyositis. Korean J Med. 2009. 77:479–83.

31). Hochberg M., Feldman D., Stevens M., Arnett F., Reichlin M. Antibody to Jo-1 in polymyositis/dermatomyositis: association with interstitial pulmonary disease. J Rheumatol. 1984. 11:663.

32). Fudman E., Schnitzer T. Dermatomyositis without creatine kinase elevation. A poor prognostic sign. Am J Med. 1986. 80:329–32.