Prevalence and clinical significance of systemic sclerosis-associated myopathy in 429 patients
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摘要: 目的 探讨系统性硬化症(systemic sclerosis,SSc)伴发肌病患者的临床特征。方法 对2009年2月~2015年12月在北京协和医院欧洲抗风湿病联盟硬皮病试验研究组(European scleroderma trials and research group,EUSTAR)数据库中收集的SSc患者的肌肉合并症进行横断面分析,比较SSc伴发肌病组和无肌病组的流行病学及临床资料,分析两组间的差异。结果 共有资料完整患者429人纳入本研究,平均发病年龄为(39.3±12.6)岁,SSc伴发肌病的发生率为22.6%。两组比较,肌病组的早饱/呕吐(χ2=16.29,P<0.001)、吞咽困难(χ2=27.01,P<0.001)、浮肿(χ2=17.74,P<0.001)及尿量减少(χ2=4.59,P=0.032)发生率高于无肌病组。SSc伴发肌病组超声心动图检查提示肺动脉压升高的发生率(χ2=6.31,P=0.012)高于无肌病组。SSc伴发肌病组的血沉(t=1.06,P=0.011)和C反应蛋白水平(t=0.87,P=0.001)高于无肌病组。结论 SSc伴发肌病不少见,与疾病的炎症程度及肾脏受累、心脏和肺并发症相关,临床需加强对SSc伴发肌病患者的关注并警惕与心脏和肾脏相关的并发症。Abstract: Objective To explore the prevalence of and clinical manifestation associated with myopathy in Chinese patients with systemic sclerosis(SSc). Methods This study was a cross-sectional study based on European Scleroderma Trials and Research group (EUSTAR) database in Peking Union Medical College Hospital(PUMCH) from February 2009 to December 2015. Demographic, clinical and laboratory data were analyzed in groups with and without myopathy. Results Of 429 Chinese SSc patients whose mean age of onset was (39.3±12.6) years old. Overall prevalence of myopathy was 22.6%. The presence of early satiety/vomiting(χ2=16.29, P<0.001), dysphagia (χ2=27.01,P<0.001), edema (χ2=17.74, P<0.001), and urine decreased(χ2=4.59, P=0.032) occurred more frequent in patients with myopathy than those without myopathy. The abnormal rate of pulmonary arterial hypertension (PAH) detected by echocardiography in the myopathy group was higher than that of non-myopathy group (χ2=6.31, P=0.012). The elevation of erythrocyte sedimentation rate (t=1.06, P=0.011) and C reactive protein (t=0.87, P=0.001) were more higher in myopathy group than in non-myopathy group. Conclusions SSc associated myopathy is not uncommon. Compared to non-myopathy patients, SSc patients associated myopathy have a more severe inflammation response, and require more attention on cardiac and renal complications.
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Key words:
- Sclerosis,systemic /
- Myopathy /
- Myositis
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