儿童局限性硬皮病的诊疗进展  

Advances in diagnosis and treatment of juvenile localized scleroderma

作　　者：张彩慧(综述) 宋红梅(审校)[1] Zhang Caihui;Song Hongmei(Department of Pediatrics,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院儿科,100730

出　　处：《国际儿科学杂志》2022年第10期708-712,共5页International Journal of Pediatrics

基　　金：国家重点研发计划(2021YFC2702000)。

摘　　要：儿童局限性硬皮病罕见,其发病机制尚未完全明确,早期诊断至关重要。早期干预与良好的预后相关,但尚缺乏相对可靠的评估方法。目前该病主要诊断依据为临床表现,对诊断存疑时,需行皮肤或皮下组织活检;高频多普勒超声、剪切波超声弹性成像、红外热成像、MRI和面部锥形束计算机断层扫描、皮肤病评估工具等可评估病情;诊治中应完善系统评估。激素及甲氨蝶呤仍是一线治疗方案,二线疗法证据有限,霉酚酸酯、羟氯喹、环孢素A、托珠单抗、英夫利昔单抗等可能发挥一定作用。Juvenile localized scleroderma is a rare childhood disease,the specific pathogenesis of this disease has not been fully clarified.Early recognition and timely diagnosis are crucial,early intervention is associated with good prognosis.But there is a lack of relatively reliable evaluation of disease activity and treatment response.At present,the main diagnostic basis of the disease is clinical manifestations.When there is doubt about the diagnosis,skin or subcutaneous tissue biopsy is required.In addition,high frequency Doppler ultrasound,shear-wave elastography,infrared thermal imaging,MRI,cone beam computed tomography,and dermatology evaluation tools,etc.,all can reflect the patient′s condition to a certain extent.However,systematic evaluation should be improved in the process of diagnosis and treatment.In terms of treatment,hormones and methotrexate are still the first-line treatment for the disease,while evidence for second-line therapy is limited.Mycophenolate,hydroxychloroquine,cyclosporine A,tocilizumab and infliximab may play a certain role in the treatment of this disease.

关 键 词：儿童 局限性硬皮病 诊断 治疗 发病机制 

分 类 号：R725.9[医药卫生—儿科]