| Two cases of Spontaneous pneumomediastinum in amyopathic dermatomyositis |
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Hee Yeon Lee, Hyung Il Moon, Kwi Young Kang, Ji Hyeon Ju, Sung Hwan Park, Ho Youn Kim |
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| 자발성 종격동기종이 동반된 근침범이 없는 피부근육염 2예 |
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이희연, 문형일, 강귀영, 주지현, 박성환, 김호연 |
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| Abstract |
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Amyopathic dermatomyositis is defined by characteristic cutaneous manifestation of dermatomyositis without evidence of muscle involvement. Spontaneous pneumomediastinum is a rare complication of dermatomyositis. Pulmonary-fibrosis and vasculitis-induced bronchial-wall necrosis is suggested as the cause of pneumomediastinum in dermatomyositis. We report two cases of amyopathic dermatomyositis with spontaneous pneumomediastinum. The patients died from respiratory failure, despite high-dose steroid and cyclophosphamide administration. Thus, if amyopathic dermatomyositis with interstitial lung disease is diagnosed, clinicians should keep in mind the risk of rapid progression of interstitial lung disease and poor prognosis, especially when spontaneous pneumomediastinum is also present. |
| Key Words:
Dermatomyositis, Pneumomediastinum |
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