Clinical Differences Between Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis and Classic Dermatomyositis
Clinical Differences Between Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis and Classic Dermatomyositis
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DOI：
10.1378/chest.08-2740
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发表时间：
2009-11-01
期刊：
影响因子：
9.6
通讯作者：
Kohno, Shigeru
中科院分区：
文献类型：
--
作者：
Mukae, Hiroshi;Ishimoto, Hiroshi;Kohno, Shigeru
背景：间质性肺病(ILD)与临床肌萎缩性皮肌炎(CADM)相关，是一种潜在的致命性疾病，其临床特征尚不清楚。本研究旨在明确CADM合并ILD(CADM-ILD)和经典型皮肌炎合并ILD(DM-ILD)患者的临床特征和预后的差异。方法：对2001年至2007年在长崎大学医院住院的CADM-ILD和经典型DM-ILD患者进行回顾性研究。研究组包括11例CADM-ILD和16例经典型DM-ILD。结果：CADM-ILD患者的PaO(2)/FIO(2)比值明显低于经典型DM-ILD患者。CADM-ILD患者BAL液中淋巴细胞亚群比例明显高于经典型DM-ILD患者。ILD分为急性或慢性，急性亚型在CADM-ILD患者中比在典型DM-ILD患者中更常见。CADM-ILD患者死亡率(45%)明显高于经典型DM-ILD患者(6%)，且所有CADM死亡均发生在CADM-ILD急性亚型患者。结论：CADM-ILD患者急性亚型ILD的发生率较高，导致CADM-ILD患者的死亡率较高。(《胸部》2009；136：1341-1347)
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).Method: 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. (CHEST 2009; 136:1341-1347)