Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis
Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis
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DOI：
10.1093/rheumatology/keh723
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发表时间：
2005-10-01
期刊：
影响因子：
5.5
通讯作者：
Song, YW
中科院分区：
文献类型：
--
作者：
Kang, EH;Lee, EB;Song, YW
目标。目的探讨韩国多发性肌炎（PM）、皮肌炎（DM）和淀粉样皮肌炎（ADM）患者间质性肺疾病（ILD）的患病率、特点及预后因素。我们回顾了1984年至2003年间在首尔国立大学医院风湿病门诊就诊的72例PM和DM患者的医疗记录，其中包括6例ADM患者。29例PM/DM患者（40.3%）发生ILD。抗jo -1抗体和关节痛与ILD的存在相关（分别为P = 0.022和P = 0.041），而吞咽困难更常见于无ILD的患者（P = 0.041）。肺活检显示弥漫性肺泡损伤（DAD） （n = 2），伴DAD的常规间质性肺炎（UIP） （n = 2）、UIP （n = 1）和非特异性间质性肺炎（n = 2）。29例患者中死亡11例（37.9%）。ILD患者的平均生存时间明显短于无ILD患者（13.8 +/- 1.8年vs 19.2 +/- 0.9年，P = 0.017）。ILD患者的低生存率与哈曼-富样表现（P = 0.0000）、ADM特征（P = 0.0001）和初始用力肺活量（FVC）相关。
Objective. To assess the prevalence, characteristics and prognostic factors of interstitial lung disease (ILD) in Korean patients with polymyositis (PM), dermatomyositis (DM) and amyopathic dermatomyositis (ADM).Methods. We reviewed the medical records of 72 consecutive PM and DM patients, including six patients with ADM, who were seen at the Rheumatology Clinic of Seoul National University Hospital between 1984 and 2003.Results. Twenty-nine PM/DM patients (40.3%) developed ILD. Anti-Jo-1 antibody and arthralgia were associated with the presence of ILD (P = 0.022 and P = 0.041, respectively), whereas dysphagia was more frequently found in patients without ILD (P = 0.041). Lung biopsies revealed diffuse alveolar damage (DAD) (n = 2), usual interstitial pneumonia (UIP) with DAD (n = 2), UIP (n = 1), and non-specific interstitial pneumonia (n = 2). Of the 29 patients, 11 (37.9%) died. The mean survival time in ILD patients was significantly shorter than in those without ILD (13.8 +/- 1.8 vs 19.2 +/- 0.9 yr, P = 0.017). Poor survival in ILD patients was associated with a Hamman-Rich-like presentation (P = 0.0000), ADM features (P = 0.0001) and an initial forced vital capacity (FVC)