Division of Rheumatic Diseases, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku, Tokyo 162-8655, Japan.
Mod Rheumatol. 2012 Apr;22(2):243-8. doi: 10.1007/s10165-011-0502-z. Epub 2011 Sep 9.
The clinical course of diffuse alveolar damage (DAD) was studied in six consecutive cases of dermatomyositis (DM) based on our hospital records over 8 years. Three patients had severe myopathy at presentation, and the other three patients showed clinically amyopathic DM (CADM). Interstitial pneumonia in all patients developed shortly after they manifested DM. DAD in five deceased patients, which was proven pathologically, did not respond to steroid therapy combined with cyclosporine or tacrolimus. Of these, two patients began receiving combination therapy before suffering respiratory symptoms, and one of them had elevated serum Krebs von der Lungen-6 (KL-6) levels before visible abnormalities appeared on a plain chest X-ray. Only one patient with CADM survived; this patient received intravenously administered pulse cyclophosphamide (IVCY) therapy intravenously for DAD from the early stage. Delayed adjunctive IVCY was ineffective for progressed DAD in the remaining five patients. Elevated serum ferritin levels were observed in all four patients examined and might have predicted the lethal DAD, as in a previous report. In conclusion, promptly beginning IVCY therapy may be beneficial for patients with DM and interstitial pneumonia who show elevated serum levels of ferritin or KL-6 with minimal pulmonary abnormalities.
根据我们医院 8 年来的病历记录，对 6 例连续的皮肌炎（DM）患者的弥漫性肺泡损伤（DAD）临床病程进行了研究。3 名患者在就诊时即有严重的肌病，另外 3 名患者表现为临床无肌病性皮肌炎（CADM）。所有患者在出现 DM 后不久均发生间质性肺炎。5 例死亡患者的 DAD 经病理证实，对联合应用激素、环孢素或他克莫司治疗均无反应。其中，2 例在出现呼吸道症状前开始接受联合治疗，1 例在胸片出现明显异常前血清 Krebs von der Lungen-6（KL-6）水平升高。仅 1 例 CADM 患者存活；该患者在 DAD 早期接受静脉注射环磷酰胺（IVCY）治疗。其余 5 例患者进展性 DAD 时加用 IVCY 无效。所有 4 例检查的患者均观察到血清铁蛋白升高，这可能与既往报告的致死性 DAD 相关。总之，对于出现血清铁蛋白或 KL-6 水平升高且肺部异常轻微的 DM 伴间质性肺炎患者，尽早开始 IVCY 治疗可能有益。
