局限性硬皮病病情评估和治疗进展

Measurement of disease severity and treatment of localized scleroderma

局限性硬皮病是一种以局限性皮肤及皮下组织纤维化为特征的疾病。目前缺乏统一的病情评估标准，有作者提出多种评估方法，如半定量评分及利用计算机进行评分，并研究了激光多普勒血流仪、超声、磁共振在该病评估中的作用。治疗主要集中在免疫调节和抗纤维化方面，近年来评估了多种药物的疗效，如他克莫司、咪喹莫特、甲氨蝶呤和波生坦等，光疗也得到广泛应用。因此，文中总结近年来局限性硬皮病病情评估和治疗方面的研究进展。

Localized scleroderma （LS） is clinically characterized by idiopathic fibrosis of localized skin and subcutaneous tissues. No reliable assessments of disease severity are available for LS. Several outcome measures have been proposed in recent publications, such as semiquantitative scoring system and computer-based scoring method. The performance of laser doppler fiowmetry, super sound, and magnetic resonance have been explored in the measurement of disease severity of LS. Recent advances in therapy of this disease mainly focus on immunomodulators and anti-fibrosis agents. Some new treatment options for LS, such as tacrolimus, imiquimod, methotrexate, bosentan, phototherapy with UVA light, have been introduced and their efficacy has been estimated. This article reviews advances in disease severity measures and therapy of LS.