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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

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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.

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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 treatment 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.

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