Shimojima Yasuhiro, Ishii Wataru, Kato Takashi, Hoshi Kenichi, Matsuda Masayuki, Hashimoto Takao, Tanaka Yukio, Ikeda Shu-ichi
Third Department of Medicine, Shinshu University School of Medicine, Matsumoto.
Intern Med. 2003 Dec;42(12):1253-8. doi: 10.2169/internalmedicine.42.1253.
We report a patient with amyopathic dermatomyositis (DM) who mainly showed interstitial pneumonia and intractable skin necrosis in bilateral elbows and soles with a poor response to immunomediated therapy, including corticosteroid and high-dose intravenous immunoglobulin. Soon after starting oral cyclosporin A (CyA) the skin lesions healed completely and the interstitial pneumonia promptly improved in parallel with a decrease in serum KL-6. Since fatal interstitial pneumonia is frequently associated with amyopathic DM as in this case, administration of CyA should be actively considered as a therapeutic option when clinical symtoms are progressive and resistant to conventional treatments.
我们报告了一例无肌病性皮肌炎（DM）患者，该患者主要表现为间质性肺炎以及双肘和足底顽固性皮肤坏死，对包括皮质类固醇和大剂量静脉注射免疫球蛋白在内的免疫介导治疗反应不佳。开始口服环孢素A（CyA）后不久，皮肤病变完全愈合，间质性肺炎也迅速改善，同时血清KL-6水平下降。由于如该病例所示，致命性间质性肺炎常与无肌病性DM相关，因此当临床症状呈进行性且对传统治疗耐药时，应积极考虑将CyA作为一种治疗选择。
