儿童硬皮病临床特点分析  

Analysis of clinical characteristics of juvenile scleroderma

作　　者：邓江红[1] 李彩凤[1] 韩彤昕[1] 王江[1] 邝伟英[1] 周怡芳[1] 张俊梅[1] 

机构地区：[1]首都医科大学附属北京儿童医院风湿免疫科,100045

出　　处：《中华风湿病学杂志》2014年第9期602-606,I0002,共6页Chinese Journal of Rheumatology

摘　　要：目的 分析儿童硬皮病临床及实验室检查特点.方法 对2002年1月到2013年10月在北京儿童医院风湿免疫科住院并诊断为儿童硬皮病的病例进行回顾性研究,按照国际上最新的硬皮病诊断标准重新进行评估,对患儿的流行病学及临床资料进行回顾性分析.定量资料2组间比较采用t检验.结果 共纳入46例患者,局限性硬皮病（LS）患者39例（85％）,SSc患者7例（15％）.LS患者平均起病年龄（5±4）岁,男女比例为1.2：1,SSc患者平均年龄（9±4）岁,均为女性.39例LS患者中,带状硬皮病21例（54％）,混合性硬皮病14例（36％）,广泛性硬斑病3例（8％）,全硬化性硬斑病1例（3％）.26例患者（67％）出现皮肤外脏器受累表现,3例神经系统受累见于创伤样硬皮病患儿.7例SSc患者均有脏器受累.以肺脏及消化道受累最多见,并可见心脏、神经系统及肾脏、眼科受累病例,1例硬皮病肾危象病例.实验室检查：30例（77％）LS患者ANA阳性,6例（15％）患者RF阳性,其中5例均有关节受累表现.7例（100％） SSc患者ANA均为高滴度阳性,5例（71％）患者Scl-70阳性.主要的治疗药物是甲氨蝶呤及泼尼松.结论 儿童局限性硬皮病更为常见,SSc引起脏器损害更重,多中心更大样本的研究有助于理解本病的全貌。Objective To describe and analyze the clinical and laboratory findings in a group of children diagnosed with scleroderma at Beijing Children＇s Hospital in the last 10 years.Methods The clinical charts of children with scleroderma in the Rheumatology Department at Beijing Children＇s Hospital,between January 2002 and October 2013 were reviewed.All of them fulfilled the classification criteria for juvenile sclerodema,both systemic scleroderma （SSc） and localized scleroderma （LS） types.T test was used for comparison between the two groups.Results Forty-six patients were enrolled and were diagnosed as scleroderma.Seven patients（15％） suffered from SSc and 39 patients（85％） were LS.Mean age-at-onset of LS was （5±4） years old.The male to female ratio was 1.2：1.Mean age-at-onset of SSc was （9±4） years old.All patients were female.The lesions found in LS were linear scleroderma （54％）,mixed morphea （36％）,generalized morphea （8％）,and panclerotic morphea （3％）.Twenty-six patients had internal organs involved.Three patients with nerve system involvement was found in en coup de sabre （ECDS）.Systemic involvement included lung and gastrointestinal tract primarily.The heart,nerve system,kidney,eye involvement was also found.One girl had SSc combined with renal crisis.Antinuclear antibodies were positive in 77％ of LS patients and 100％ of SSc patients.Rheumatic factor was positive in 6 patients （15％）,5 patients had joint involvement.Tests for anti-Scl-70 antibodies were positive in 5 （71％） patients with SSc.The most common drugs used were methotrexate and prednisone.Conclusion In this study,LS is common in children.SSc is more severe than LS.Multi-center and large sample study is needed to know the characteristics of juvenile scleroderma in China.

关 键 词：儿童 硬皮病 局部性 硬皮病 系统性 

分 类 号：R725.9[医药卫生—儿科]