Tokiyama K, Tagawa H, Yokota E, Nagasawa K, Kusaba T, Tsuda Y, Niho Y
First Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka-city.
Ryumachi. 1990 Jun;30(3):204-9; discussion 209-11.
Two middle-aged women showed typical erythematous heliotrope eruption and Gottron's sign without any symptom of myositis. The patients were diagnosed as 'amyopathic dermatomyositis' because of normal serum CPK levels, normal EMG and no histological abnormality by muscle biopsy. Clinical manifestations improved by the treatment with corticosteroids. During tapering of corticosteroids, however, intersititial pneumonitis developed and rapidly progressed. The first patients was treated with methylprednisolone pulse therapy, azathiopurine and methotrexate. The second patients was treated with betamethazone, methlprednisolone pulse therapy and cyclosporin A. In spite of these extensive immunosuppressive therapies, both patients died of pulmonary insufficiency a few months after admission. In the literature there has been only several cases of amyopathic dermatomyositis and only one case with fatal rapidly progressive interstitial pneumonitis. A new approach to the treatment of this disease should be made.
两名中年女性表现出典型的紫红色向阳疹和Gottron征，但无任何肌炎症状。由于血清肌酸磷酸激酶（CPK）水平正常、肌电图正常且肌肉活检无组织学异常，这两名患者被诊断为“无肌病性皮肌炎”。使用皮质类固醇治疗后临床表现有所改善。然而，在逐渐减少皮质类固醇剂量的过程中，间质性肺炎出现并迅速进展。第一名患者接受了甲泼尼龙冲击疗法、硫唑嘌呤和甲氨蝶呤治疗。第二名患者接受了倍他米松、甲泼尼龙冲击疗法和环孢素A治疗。尽管采取了这些广泛的免疫抑制治疗措施，但两名患者在入院后几个月均死于肺功能不全。文献中仅有几例无肌病性皮肌炎病例，且仅有一例死于快速进展的致命性间质性肺炎。应对这种疾病的治疗方法进行新的探索。
