嗜酸性筋膜炎与系统性硬化症临床特点比较

目的回顾性分析嗜酸性筋膜炎(eosinophilic fasciitis,EF)患者的临床特点,并与系统性硬化症(systemic sclerosis,SSc)进行比较,提高对EF的认识。方法收集山西大医院和山西医科大学第二医院2002年5月至2013年5月EF住院患者资料,以及2011年12月至2014年8月山西大医院确诊为SSc的住院患者资料,对其临床特点、实验室检查及治疗与转归等进行比较。结果 EF男性多见而SSc女性多见(P0.001);EF组年龄和病程均低于SSc组(P0.01);且EF组发病前有明确诱因5例(42%),而SSc组发病前均无明确诱因。以雷诺现象为首发症状者在SSc组(54%,1324)显著高于EF组(0%),而EF患者多同时伴有明显疼痛(50%,612)(P0.05)。EF组皮肤及脏器受累均显著低于SSc组(P0.05)。EF组患者(58%,712)嗜酸粒细胞较SSc组(0%)显著升高(P0.05),而抗核抗体阳性率则明显降低(P0.05)。EF组病例用泼尼松治疗效果显著,预后较SSc好。结论 EF与SSc在一般情况、临床特点、实验室检查等方面均有明显差异,EF可能为一种不同于SSc的独立性疾病。

Comparison of Clinical Characteristics between Eosinophilic Fasciitis and Systemic Sclerosis

Objective To improve the awareness of eosinophilic fasciitis （EF） through a retrospectively analysis of clinical characteristics of patients with EF and a comparison with systemic sclerosis （SSc）.Methods Information of patients,who were diagnosed as EF and hospitalized in Shanxi Dayi Hospital and the Second Hospital of Shanxi Medical University from May 2002 to May 2013 and who were diagnosed as SSc and hospitalized in Shanxi Dayi Hospital from December 2011 to August 2014,were collected.The data of clinical features,laboratory tests results and treatments were collected and compared.Results Compared with the SSc,EF was male predominant （P ＜ 0.001）,and was remarkablely lower than SSc in age and disease course （P ＜ 0.05）.There were 5 cases （42％） with predisposing factors before the onset in the EF group,but no one in the SSc group.Raynaud＇s phenomenon as the initial symptoms in SSc （54％,13/24） was significantly higher than that in EF （0％）,but EF patients experienced more severe pain （50％,6/12） （P ＜ 0.05）.Compared with the EF group,SSc group had higher prevalence of skin and organs involvement （P ＜ 0.05）.EF （58％,7/12） was remarkable higher than SSc （0％） in the eosinophil count,but was remarkablely lower in the antinuclear antibody （P ＜ 0.05）.Prednisone treatment was very effective in EF,and the prognosis was better than SSc.Conclusions Between EF and SSc,There are significant difference in clinical features,laboratory test results and treatments.Therefore,EF and SSc maybe two independent diseases.