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无肌病性皮肌炎与典型皮肌炎相关间质性肺病有差别

上一篇 / 下一篇 2012-01-29 09:33:33 / 个人分类：多肌炎/皮肌炎专题

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刘蕊

摘要（日本）背景:临床上，无肌病性皮肌炎(CADM)相关间质性肺病（ILD）有潜在致死风险，且临床特点并不十分清楚。因此，我们研究的目的是来明确CADM相关ILD(CADM-ILD)和典型皮肌炎相关ILD(DM-ILD)临床表现及预后的差别。

方法:我们回顾性研究了自2001至2007年在长崎大学医院住院的CADM-ILD 和典型的 DM-ILD患者。共纳入CADM-ILD 患者11例，典型 DM-ILD患者16例。并对两组患者的临床特征和预后进行比较。

结果: CADM-ILD 组Pao(2)/Fio(2)率显著低于典型的 DM-ILD组，CADM-ILD 组支气管镜灌洗液淋巴细胞亚群比例显著高于典型的 DM-ILD组。ILD分为急性和慢性，急性型更易出现在CADM-ILD组，CADM-ILD组的死亡率是45%，显著高于DM-ILD组6%的死亡率，且CADM-ILD组患者中所有死亡的患者均为急性CADM-ILD亚型者。

结论: 与典型皮肌炎相比，无肌病性皮肌炎发生急性间质性肺病较高，并导致其高死亡率。

附原文： Abstract BACKGROUND: Interstitial lung disease (ILD) associated with clinically amyopathic dermatomyositis (CADM) is a potentially fatal condition in which the clinical features are not well understood. The aim of the present study was to clarify the differences in clinical characteristics and prognosis of patients with ILD associated with CADM (CADM-ILD) and classic dermatomyositis associated with ILD (DM-ILD). METHODS: We retrospectively studied consecutive patients with CADM-ILD and classic DM-ILD who were hospitalized between 2001 and 2007 at Nagasaki University Hospital. The study group consisted of 11 patients with CADM-ILD and 16 patients with classic DM-ILD. We compared the clinical features and prognosis between the two forms. RESULTS: The Pao(2)/Fio(2) ratio was significantly lower in patients with CADM-ILD than in patients with classic DM-ILD. The lymphocyte subsets ratio in the BAL fluid of patients with CADM-ILD was significantly higher than the corresponding ratio in patients with classic DM-ILD. ILD is classified as acute or chronic, and the acute subtype was more common in patients with CADM-ILD than in those with classic DM-ILD. The mortality rate for patients with CADM-ILD (45%) was much higher than that for patients with classic DM-ILD (6%), and all of the CADM deaths occurred in the group of patients with acute CADM-ILD. CONCLUSION: Our data suggest that the higher prevalence of the acute subtype of ILD in patients with CADM results in a higher mortality rate for patients with CADM-ILD.

引自: Mukae H, Ishimoto H, Sakamoto N, Hara S, Kakugawa T, Nakayama S, Ishimatsu Y, Kawakami A, Eguchi K, Kohno S. Clinical differences between interstitial lung disease associated with clinically amyopathic dermatomyositis and classic dermatomyositis. Chest, 2009,136(5):1341-7.