Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study
Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study
复制标题
DOI：
10.1007/s10067-007-0562-9
复制
发表时间：
2007-10-01
影响因子：
3.4
通讯作者：
Chen, Shun-le
中科院分区：
文献类型：
--
作者：
Ye, Shuang;Chen, Xiao-xiang;Chen, Shun-le
本研究旨在探讨成人临床无肌病性皮肌炎（CADM）伴快速进展性间质性肺病（ILD）的特点。对1998 ~ 2005年上海仁济医院皮肌炎和多发性肌炎住院患者的临床资料进行回顾性分析。145例患者根据改良Sontheimer定义或Bohan-Peter分类标准分为CADM、经典DM和PM。根据是否存在临床ILD对其进行进一步分层。采用Kaplan-Meier生存分析和考克斯回归分析。探讨ILD的预测因素和CADM-ILD的其他临床特征。临床ILD的存在是DM/PM不良结局的显著风险因素（OR=4.237，CI 95%：1.239-14.49，p=0.021）。其他危险因素是皮疹和尿素氮升高。DM/PM合并ILD患者的临床病程不同。CADM-ILD患者表现为快速进展模式，6个月生存率为40.8%。DM-ILD表现为进行性模式，5年生存率为54%，而PM-ILD为慢性，5年和10年生存率分别为72.4%和60.3%。DM/PM患者肌肉力量保存较好、红细胞沉降率升高和低白蛋白血症可能预示ILD。与存活者相比，后来死亡的CADM-ILD患者的PO 2较低，乳酸脱氢酶较高，关节炎/关节痛突出。抗核抗体的存在似乎是保护性的。快速进展的CADM-ILD对常规治疗难治。ILD是我们超过40%的住院DM/PM队列的常见并发症，也是一个突出的预后指标。CADM是DM/PM的一种特殊表型。CADM-ILD通常是快速进展和致命的，需要进一步研究。
The aim of the study was to investigate the characteristics of adult clinically amyopathic dermatomyositis (CADM) with rapid progressive interstitial lung disease (ILD). Hospitalized patients with dermatomyositis (DM) and polymyositis (PM) between 1998 and 2005 in the Shanghai Renji Hospital were retrospectively studied. One hundred and forty-five patients were classified into CADM, classic DM or PM according to the modified Sontheimer's definition or Bohan-Peter's classification criteria. They were further stratified based on the presence or absence of clinical ILD. The Kaplan-Meier survival analysis and COX regression were performed. The predictive factors for ILD and other clinical properties of CADM-ILD were explored. The presence of clinical ILD was a significant risk factor for the poor outcome of DM/PM (OR=4.237, CI 95%: 1.239-14.49, p=0.021). Other risk factors are the presence of rashes and elevated urea nitrogen. Patients with DM/PM complicated by ILD had different clinical courses. Patients with CADM-ILD showed a rapidly progressive pattern with 6-month survival rate of 40.8%. The DM-ILD manifested a progressive pattern with a 5-year survival rate of 54%, while PM-ILD was chronic with 5- and 10-year survival rate of 72.4% and 60.3%, respectively. Better preserved muscle strength, elevated erythrocyte sedimentation rate, and hypoalbuminemia may herald ILD in DM/PM. Patients with CADM-ILD who later died had lower PO2, higher lactate dehydrogenase, and prominent arthritis/arthralgia compared with those who survived. The presence of antinuclear antibody seems to be protective. Rapid progressive CADM-ILD is refractory to conventional treatment. ILD is a common complication in over 40% of our hospitalized DM/PM cohort and is also a prominent prognostic indicator. CADM is a special phenotype of DM/PM. CADM-ILD, which is usually rapidly progressive and fatal, requires further investigation.