Longterm Survival and Associated Risk Factors in Patients with Adult-onset Idiopathic Inflammatory Myopathies and Amyopathic Dermatomyositis: Experience in a Single Institute in Japan
Longterm Survival and Associated Risk Factors in Patients with Adult-onset Idiopathic Inflammatory Myopathies and Amyopathic Dermatomyositis: Experience in a Single Institute in Japan
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DOI：
10.3899/jrheum.101002
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发表时间：
2011-08-01
影响因子：
3.9
通讯作者：
Ozaki, Shoichi
中科院分区：
文献类型：
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作者：
Yamasaki, Yoshioki;Yamada, Hidehiro;Ozaki, Shoichi
Objective.分析日本成人发病的特发性炎性肌病（IIM）患者的临床特征、生存率、死亡原因以及与死亡率相关的危险因素。我们回顾性调查了1984年至2009年在我院确诊的197例成人发病的IIM患者，根据Bohan和Peter的多发性肌炎（PM）/皮肌炎（DM）标准和改良Sontheimer的临床无肌病性DM（ADM）标准。整个组的1年、5年和10年生存率分别为85%、75%和67%。癌症相关性肌炎的死亡率最高（5年时为25%），其次是临床ADM（5年时为61%）和原发性DM（5年时为77%）。与原发性PM相比，原发性DM的生存率显著较低（5年时为91%; p = 0.0427）。死亡的53例患者中，ADM患者6例（11%），原发性DM患者20例（38%）。间质性肺疾病（ILD）是临床ADM（71%）和原发性DM（60%）的主要死亡原因，大多数发生在最初几个月内。较少的患者死于原发性PM（9%）和重叠性肌炎（13%）。死亡的独立危险因素是年龄较大（HR 1.031; 95%CI 1.009-1.053）和皮肤溃疡（HR 3.018; 95%CI 1.340-6.796），原发性DM患者中ILD伴轻度血清肌酸激酶水平（< 500 IU/l; HR 3.537; 95%CI 1.260-9.928）。与原发性PM相比，临床ADM和原发性DM的生存率较低，主要是由于致死性ILD。确立ILD的治疗策略可能会提高我们患者人群的生存率。（2011年5月15日首次发布; J Rheumol 2011;38：1636-43; doi：10.3899/jrheum.101002）
Objective. To analyze clinical characteristics, survival, causes of death, and risk factors associated with mortality in patients with adult-onset idiopathic inflammatory myopathies (IIM) in Japan.Methods. We retrospectively investigated 197 patients diagnosed with adult-onset IIM at our hospital from 1984 to 2009 according to Bohan and Peter criteria for polymyositis (PM)/dermatomyositis (DM) and modified Sontheimer's criteria for clinically amyopathic DM (ADM).Results. Survival in the whole group at 1, 5, and 10 years was 85%, 75%, and 67%, respectively. Mortality in cancer-associated myositis was the worst (25% at 5 yrs), followed by clinically ADM (61% at 5 yrs) and primary DM (77% at 5 yrs). Primary DM had significantly low survival compared to primary PM (91% at 5 yrs; p = 0.0427). Among the 53 patients who died were 6 patients with ADM (11%) and 20 patients with primary DM (38%). Interstitial lung disease (ILD) was the main cause of death in clinically ADM (71%) and primary DM (60%), most of which occurred within the first few months. Fewer patients died in primary PM (9%) and overlap myositis (13%). Independent risk factors for death were older age (HR 1.031; 95% CI 1.009-1.053) and skin ulcers (HR 3.018; 95% CI 1.340-6.796) in the whole group and ILD with mild serum creatine kinase levels (< 500 IU/l; HR 3.537; 95% CI 1.260-9.928) in primary DM.Conclusion. Survival of clinically ADM and primary DM was low, mainly due to fatal ILD, compared to primary PM. Establishing therapeutic strategy for ILD may improve the survival in our patient population. (First Release May 15 2011; J Rheumatol 2011;38:1636-43; doi:10.3899/jrheum.101002)