皮肌炎相关间质性肺病与特发性肺纤维化临床特征与预后的差异  

Difference of the clinical characteristics and outcome between interstitial lung disease with dermatomyositis and idiopathic pneumonia fibrosis

作　　者：冯定云[1] 邹小玲[1] 杨海玲[1] 李和亮 周宇麒[1] 张天托[1] Feng Dingyun;Zou Xiaoling;Yang Hailing;Li Heliang;Zhou Yuqi;Zhang Tiantuo(Department of Respiratory Disease,The Third Affiliated Hospital of Sun Yat-sen University,Guangzhou 510630,China;The Zhongshan School of Medicine,Sun Yat-sen University,Guangzhou 510000,China)

机构地区：[1]中山大学附属第三医院呼吸内科,广州510630 [2]中山大学中山医学院,广州510000

出　　处：《中国医师杂志》2018年第10期1498-1501,共4页Journal of Chinese Physician

摘　　要：目的比较皮肌炎相关间质性肺病(DM-ILD)与特发性肺纤维化(IPF)之间的临床特征、预后的差异。方法收集中山大学附属第三医院2003年1月至2014年3月住院患者中诊断为DM-ILD或者IPF的病例,比较其临床特征与预后的差异。结果共入组64例患者,其中DM-ILD 44例,IPF 20例。IPF多见于年长者(P=0. 000)、男性(P=0. 004)及吸烟者(P=0. 000),其高分辨CT多表现为网格状影(P=0. 014)和蜂窝状影(P=0. 000),分布较为弥漫。DM-ILD高分辨CT多表现为斑片状影(P=0. 048)和条索状影(P=0. 000),在治疗上常用糖皮质激素(P=0. 000)及免疫抑制剂(P=0. 000)。但两者的90 d病死率差异无统计学意义(P> 0. 05)。结论 IPF多见于年长者、男性及吸烟者,其高分辨CT多表现为网格状影和蜂窝状影,分布较为弥漫。DM-ILD高分辨CT多表现为斑片状影和条索状影。在治疗上,DM-ILD常用糖皮质激素及免疫抑制剂,但两者90 d死亡率无明显差异。Objective To compare the clinical features and prognosis between dermatomyositis-associated interstitial lung disease （DM-ILD） and idiopathic pulmonary fibrosis （IPF）. Methods Patients with interstitial lung disease with dermatomyositis （DM-ILD） or idiopathic pneumonia fibrosis （IPF） from January 2003 to March 2014 in the third affiliated hospital of Sun Yat-sen University were included. Results Among the 64 patients enrolled, 44 were DM-ILD and 20 were IPF. IPF was more common in the elderly （ P = 0. 000 ） , men （ P = 0. 004 ） and smokers （ P = 0. 000 ） , and its high-resolution computed tomography （CT） mostly showed grid shadow （P =0. 014） and honeycomb shadow （P =0. 000）. DM-ILD usually had cough symptoms （P = 0. 025 ）. High-resolution CT showed patchy （P =- 0. 048） and banded （P = 0. 000）. Glucocorticoid （ P = 0. 000） and immunosuppressive agents （ P = 0. 000） were commonly used in the treat- ment of DM-ILD. However, there was no significant difference in 90d mortality between the two groups （P 〉 0. 05）. Conclusions IPF is more common in the elderly, men and smokers, and its high-resolution CT mostly shows grid shadow and honeycomb shadow, distribution is diffuse. DM-ILD often has cough symptoms, and its high resolution CT is mostly plaques and streaky shadows. Glucocorticoids and immunesuppressants are commonly used in DM-ILD, but there is no significant difference in 90-day mortality between them.

关 键 词：皮肌炎/并发症 肺疾病 间质性/病因学/诊断 肺纤维化/诊断 诊断 鉴别 

分 类 号：R563[医药卫生—呼吸系统]