临床无肌病皮肌炎10例临床分析

目的:探讨临床无肌病皮肌炎(clinically amyopathic dermatomyositis,CADM)的临床特征及预后。方法:对10例CADM患者的皮肤、肌肉损害以及肺部病变等临床资料进行回顾性分析。结果:10例CADM患者均出现皮肌炎特征性的皮肤改变,其中Gottron疹9例,向阳疹8例,颈部V区疹10例,披肩疹5例。9例患者合并肺间质病变,其中4例在随访中病死,主要病死原因为合并肺间质病变进展至呼吸衰竭。结论:肺间质病变是CADM最常见和最严重的并发症,也是病死的主要原因,临床上须高度警惕,一旦发现应积极治疗。

Analysis of 10 cases of clinically amyopathic dermatomyositis

Objective：To investigate the clinical features and prognosis of clinically amyopathic dermatomyositis（CADM）.Methods：The clinical data including the skin lesion,muscle involvement and pathological changes of the lung in 10 patients with CADM were analyzed retrospectively.Results：All the 10 patients presented characteristic skin lesions such as Gottron,S papulae（9 cases）,heliotropic periorbital erythrema（8 cases）,V-sign rash（10 cases） and shawl-sign rash（5 cases）.Interstitial lung disease（ILD） occurred in 9 patients,and 4 of them died of respiratory failure resulting from rapidly progressive ILD.Conclusions：ILD is the most common and severe complication leading to mortality in CADM patients;therefore it should be highly alerted to and properly managed.