Department of Internal Medicine, Toho University Sakura Medical Center, Shimoshizu, Sakura, Japan.
Respirology. 2010 Nov;15(8):1261-5. doi: 10.1111/j.1440-1843.2010.01851.x.
Pulmonary fibrosis associated with amyopathic dermatomyositis is known to have a generally aggressive course and is ultimately fatal. We report the case of a 50-year-old patient with amyopathic dermatomyositis, who developed progressive interstitial pneumonia that was unresponsive to corticosteroids and multiple immunosuppressive agents, including cyclosporine and tacrolimus hydrate. Five courses of lecithinized superoxide dismutase were administered without adverse effects. Improvements in physiological parameters, such as pulmonary function and exercise tolerance, as well as the serum Krebs von den Lungen 6 level, were observed. This is the first report of a case of steroid-refractory interstitial pneumonia treated with lecithinized superoxide dismutase.
与无肌病性皮肌炎相关的肺纤维化已知具有普遍侵袭性的病程,最终是致命的。我们报告了一例 50 岁的无肌病性皮肌炎患者,其发生进行性间质性肺炎,对皮质类固醇和多种免疫抑制剂(包括环孢素和他克莫司水合物)均无反应。给予 5 个疗程的卵磷脂超氧化物歧化酶,无不良反应。观察到肺功能和运动耐量等生理参数以及血清 Krebs von den Lungen 6 水平的改善。这是首例用卵磷脂超氧化物歧化酶治疗皮质类固醇耐药性间质性肺炎的病例报告。