A Case of Nonspecific Interstitial Pneumonia Complicated with Spontaneous Pneumomediastinum, Subcutaneous Emphysema and Pneumatosis Interstinalis

비특이성 간질성 폐렴에서 발생한 자발성 종격동기종, 피하기종과 대장기종

  • Park, Myung Jae (Department of Pulmonary and Critical Care Medicine, Kyung Hee University School of Medicine)
  • 박명재 (경희대학교 의과대학 내과학교실)
  • Received : 2008.01.14
  • Accepted : 2008.01.24
  • Published : 2008.02.28

Abstract

Pneumatosis intestinalis or spontaneous pneumomediastinum are rarely associated with nonspecific interstitial pneumonia (NSIP). However, the development of both conditions in the same patient simultaneously has not been reported previously. A 56-year-old man with NSIP developed spontaneous pneumomediastinum accompanied by subcutaneous emphysema and pneumatosis intestinalis after the treatment with intravenous high dose steroid. The development of spontaneous pneumomediastinum, subcutaneous emphysema and pneumatosis intestinalis in this patient was possibly due to the factors such as NSIP, high dose steroid therapy and subclinical dermatomyositis. Treatment with corticosteroid and cyclosporin gradually improved his exacerbated NSIP and pneumomediastinum, subcutaneous emphysema, pneumatosis intestinalis.

특징적인 피부병변, 근력약화 등 분명한 피부근염의 임상소견을 보이지 않는 환자에서 동반된 NSIP 치료경과 중 자발성 종격동기종, 피하기종 및 대장기종이 발생한 증례를 경험하였고 스테로이드와 cyclosporin을 사용하여 좋은 치료반응을 경험하였다. 간질성폐렴을 동반한 피부근염에서 자발성 종격동기종, 피하기종 또는 대장기종 등이 드물게 보고되었고 이 경우 나쁜 예후를 보이므로 주의할 필요가 있다. 비교적 드물지만 NSIP에서 종격동기종, 대장기종이 발생하는 경우 임상경과 중 피부근염이 동반 또는 이환되었는지 세심한 관찰이 필요하겠다. 또 NSIP에서 기종이 발견되는 경우 스테로이드와 cyclosporin 또는 다른 면역억제제의 병용투여 등 적극적인 치료가 치료반응과 예후에 좋은 영향을 주리라 사료되어 본 증례를 보고하는 바이다.

Keywords

References

  1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002;165:277-304 https://doi.org/10.1164/ajrccm.165.2.ats01
  2. Douglas WW, Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, et al. Polymyositis-dermatomyositis- associated interstitial lung disease. Am J Respir Crit Care Med 2001;164:1182-5 https://doi.org/10.1164/ajrccm.164.7.2103110
  3. 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 https://doi.org/10.1007/s10067-002-0675-0
  4. Wada Y, Murayama N, Hirose S, In H, Kuroda T, Ito S, et al. A case of pneumatosis cystoides intestinalis in a patient with polymyositis and interstitial pneumonia. Mod Rheumatol 2004;14:260-3 https://doi.org/10.3109/s10165-004-0304-7
  5. Won Huh J, Soon Kim D, Keun Lee C, Yoo B, Bum Seo J, Kitaichi M, et al. Two distinct clinical types of interstitial lung disease associated with polymyositis-dermatomyositis. Respir Med 2007;101:1761-9 https://doi.org/10.1016/j.rmed.2007.02.017
  6. Fujisawa T, Suda T, Nakamura Y, Enomoto N, Ide K, Toyoshima M, et al. Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis. J Rheumatol 2005;32:58-64
  7. Cottin V, Thivolet-Bejui F, Reynaud-Gaubert M, Cadranel J, Delaval P, Ternamian PJ, et al. Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis. Eur Respir J 2003;22:245-50 https://doi.org/10.1183/09031936.03.00026703
  8. Neves Fde S, Shinjo SK, Carvalho JF, Levy-Neto M, Borges CT. 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 https://doi.org/10.1007/s10067-005-0109-x
  9. Panacek EA, Singer AJ, Sherman BW, Prescott A, Rutherford WF. Spontaneous pneumomediastinum: clinical and natural history. Ann Emerg Med 1992;21:1222-7 https://doi.org/10.1016/S0196-0644(05)81750-0
  10. 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 https://doi.org/10.1080/03009749950155805
  11. Kono H, Inokuma S, Nakayama H, Suzuki M. Pneumomediastinum in dermatomyositis: association with cutaneous vasculopathy. Ann Rheum Dis 2000;59:372-6 https://doi.org/10.1136/ard.59.5.372
  12. Hisamoto A, Mizushima T, Sato K, Haruta Y, Tanimoto Y, Tanimoto M, et al. Pneumatosis cystoides intestinalis after alpha-glucosidase inhibitor treatment in a patient with interstitial pneumonitis. Intern Med 2006;45:73-6 https://doi.org/10.2169/internalmedicine.45.1330
  13. St Peter SD, Abbas MA, Kelly KA. The spectrum of pneumatosis intestinalis. Arch Surg 2003;138:68-75 https://doi.org/10.1001/archsurg.138.1.68
  14. Selva-O'Callaghan A, Martinez-Costa X, Solans-Laque R, Mauri M, Capdevila JA, Vilardell-Tarres M. Refractory adult dermatomyositis with pneumatosis cystoides intestinalis treated with infliximab. Rheumatology (Oxford) 2004;43:1196-7 https://doi.org/10.1093/rheumatology/keh285
  15. Miyake S, Ohtani Y, Sawada M, Inase N, Miyazaki Y, Takano S, et al. Usefulness of cyclosporine A on rapidly progressive interstitial pneumonia in dermatomyositis. Sarcoidosis Vasc Diffuse Lung Dis 2002;19:128-33