临床无肌病皮肌炎10例临床分析

    Analysis of 10 cases of clinically amyopathic dermatomyositis

    • 摘要: 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。

       

      Abstract: 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.

       

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