住院死亡特发性炎性肌病患者临床特点分析  

Clinical analysis of in-hospital mortality in patients with idiopathic inflammatory myopathies

作　　者：王沁 梁钧昱 孙传银[2] 余叶 曹恒[2] 林进[2] Wang Qin;Liang Junyu;Sun Chuanyin;Yu Ye;Cao Heng;Lin Jin(Division of Endocrinology and metabolism,Jinhua Municipal Central Hospital,Jinhua 321000,China;Division of Rheumatology and Immunology,the First Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310003,China)

机构地区：[1]浙江省金华市中心医院内分泌代谢科,321000 [2]浙江大学医学院附属第一医院风湿免疫科,杭州310003

出　　处：《中华风湿病学杂志》2020年第10期670-675,共6页Chinese Journal of Rheumatology

摘　　要：目的探讨住院死亡特发性炎性肌病(IIM)患者的临床特点并分析死亡的危险因素。方法回顾性分析2011年2月至2019年2月于浙江大学医学院附属第一医院住院多发性肌炎(PM)、典型皮肌炎(CDM)和临床无肌病性皮肌炎(CADM)患者的病例资料,按住院期间或出院后2周内是否死亡将患者分为住院死亡组和住院存活组,2组患者按性别年龄匹配。分析比较2组患者临床特点,采用单因素和多因素Logistic回归模型分析IIM(或亚组)患者住院死亡的危险因素。结果浙江大学医学院附属第一医院PM、CDM和CADM患者(424例)的住院死亡率为9.4%。与住院存活组患者(80例)相比,住院死亡组(40例)中位住院时间[0.9(0.5,1.0)个月与0.6(0.4,1.0)个月,Z=-2.159,P<0.05]更长;血清铁蛋白[1170.8(757.6,3759.9)μg/L与374.9(182.1,993.4)μg/L,χ2=-4.665]、红细胞分布宽度[15.2(14.5,16.3)%与14.4(13.5,15.2)%,Z=-3.066]、CRP[11.3(4.4,36.9)mg/L与5.1(1.8,17.2)mg/L,Z=-2.667]、中性粒细胞/淋巴细胞比值[10.1(5.5,18.9)与4.2(2.6,6.5),Z=-5.108]均高于存活组(P<0.05);血CEA(45.0%与12.5%,χ2=15.745)、谷氨酰胺转肽酶(55.0%与23.8%,χ2=11.578)和α-L-岩藻糖苷酶(35.0%与10.0%,χ2=10.902)升高的患者比例,以及合并ILD进展(60.0%与16.3%,χ2=23.934)、肺部感染(72.5%与20.0%,χ2=31.360)、噬血细胞综合征(35.0%与1.3%,χ2=27.771)及低T3综合征(50.0%与17.5%,χ2=16.644)的患者比例更高(P<0.05)。住院死亡患者更常用激素冲击及静脉注射用免疫球蛋白治疗。入院时高疾病活动度[OR=1.593,95%CI(1.255,2.022),P<0.001]、合并ILD进展[OR=5.600,95%CI(1.510,20.772),P=0.010]及肺部感染[OR=6.771,95%CI(2.031,22.574),P=0.002]是IIM患者住院死亡独立危险因素。在亚组分析中,肺部感染和呼吸衰竭是合并ILD进展IIM患者短期预后不良因素;向阳疹、ILD进展及接受激素加量治疗是伴肺部感染IIM患者住院死亡的危险因素。结论入院时高疾病活动度、肺部感染及ILD进展是IObjective To explore the clinical features and risk factors of in-hospital mortality in idiopathic inflammatory myopathies (IIM) patients.Results We retrospectively analyzed clinical records of polymyositis (PM), classic dermatomyositis (CDM) and clinically amyopathic dermatomyositis (CADM) patients admitted to the First Affiliated Hospital of Zhejiang University from February 2011 to February 2019. The deceased group was defined as the patients who died in hospital or within 2 weeks after hospital discharge, while the survival group was defined as the survival patients. The clinical features were described. Risk factors for deceased patients were identified by logistic regression analysis.Results The in-hospital mortality rate of IIM patients (n=424) was 9.4%. The hospitalization time was longer in deceased group (n=40) [0.9(0.5, 1.0) m vs 0.6(0.4, 1.0) m, Z=-2.159, P<0.05]. Ferritin [1170.8(757.6, 3 759.9) μg/L vs 374.9(182.1, 993.4) μg/L, Z=-4.665], red blood cell distribution width (RDW) [15.2(14.5, 16.3)% vs 14.4(13.5, 15.2)%, Z=-3.066], CRP con-centrations [11.3(4.4, 36.9) mg/L vs 5.1(1.8, 17.2) mg/L, Z=-2.667] and neutrophil-to-lymphocyte ratio (NLR) [10.1(5.5, 18.9) vs 4.2(2.6, 6.5), Z=-5.108] were higher in deceased group (P<0.05). Proportion of patients with high levels of CEA (45.0% vs 12.5%, χ2=15.745), glutamyl transpeptidase (γ-GT) (55.0% vs 23.8%, χ2=11.578), fucosidase (AFU) (35.0% vs 10.0%, χ2=10.902) and with complications [including pro-gressive in-terstitial lung disease (ILD) (60.0% vs 16.3%, χ2=23.934), pulmonary infection (72.5% vs 20.0%, χ2=31.360), hemophagocytic lymphohistiocytosis (35.0% vs 1.3%, χ2=27.771) and low T3 syndrome (50.0% vs 17.5%, χ2=16.644) were higher in deceased group (P<0.05). Steroid pulse therapy and intravenous immuno-globulin therapy were more common in deceased group. Higher on-admission disease activity [OR=1.593, 95%CI(1.255, 2.022), P<0.001], progressive ILD [OR=5.600, 95%CI(1.510, 20.772), P=0.010] and pulmonary infection [OR=6.771, 95%CI(2.031, 22.57

关 键 词：皮肌炎 多发性肌炎 无肌病性皮肌炎 医院死亡 危险因素 

分 类 号：R593.26[医药卫生—内科学]