Chaowattanapanit Suteeraporn, Choonhakarn Charoen, Foocharoen Chingching, Julanon Narachai
Division of Dermatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
Division of Allergy-Immunology-Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
Photodermatol Photoimmunol Photomed. 2017 Nov;33(6):296-305. doi: 10.1111/phpp.12331. Epub 2017 Aug 6.
Systemic scleroderma-also known as systemic sclerosis (SSc)-is a chronic systemic connective tissue disease characterized by collagen deposition in cutaneous and internal organs, leading to skin sclerosis and multiple organ fibrosis. The pathogenesis is complex and remains poorly understood. Treatment is based on organ involvement and requires a multidisciplinary approach. Skin sclerosis can cause disability, leading to decreasing quality of life. Various systemic antifibrotic therapies have been used; however, most have unsatisfactory results. Recently, phototherapy and in particular ultraviolet A (UVA) has been used to treat skin sclerosis in SSc patients with satisfactory results. The main mechanisms include lymphocyte apoptosis, cytokine alteration, inhibition of collagen synthesis and increased collagenase production, and neovascularization, leading to the breakdown of collagen fibrils resulting in skin softening or even healing digital ulcers. Most studies reported that psoralen plus UVA (PUVA) and UVA1 phototherapy improved clinical outcomes vis-à-vis skin sclerosis, joint mobility, ulcers, and histopathology. PUVA and UVA1 phototherapy therefore have potential as an alternative or adjunctive therapy for patients with SSc.
系统性硬化症——也称为系统性硬皮病（SSc）——是一种慢性全身性结缔组织疾病，其特征是皮肤和内脏器官中有胶原蛋白沉积，导致皮肤硬化和多器官纤维化。其发病机制复杂，仍知之甚少。治疗基于器官受累情况，需要多学科方法。皮肤硬化会导致残疾，从而降低生活质量。已经使用了各种全身性抗纤维化疗法；然而，大多数效果并不理想。最近，光疗尤其是紫外线A（UVA）已被用于治疗SSc患者的皮肤硬化，效果令人满意。主要机制包括淋巴细胞凋亡、细胞因子改变、抑制胶原蛋白合成和增加胶原酶产生，以及新生血管形成，导致胶原纤维分解，从而使皮肤软化甚至治愈指端溃疡。大多数研究报告称，补骨脂素加UVA（PUVA）和UVA1光疗在皮肤硬化、关节活动度、溃疡和组织病理学方面改善了临床结果。因此，PUVA和UVA1光疗有潜力作为SSc患者的替代或辅助治疗方法。
