Tratenberg Mark, Gutwein Farrah, Rao Varuni, Sperber Kirk, Wasserrman Amy, Ash Julia
Division of Allergy, Immunology, and Rheumatology, New York Medical College, Valhalla, NY, United States.
Curr Rheumatol Rev. 2017;13(2):86-92. doi: 10.2174/1573397112666160907105434.
Localized scleroderma (LS) is characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissue or both. The outcome for most patients with localized scleroderma is directly related to the type and stage of the affected tissue. The major challenge for untreated patients is not increased mortality risk, rather deformity and growth defects from skin, muscle and bone abnormalities. Treatment is individualized to type and stage of the lesion and may include pharmacologic and non-pharmacologic therapies. Among the pharmacologic modalities, methotrexate with systemic glucocorticoids is currently the mainstay of treatment. More controlled trials are needed to determine the length of treatment and the maintenance dose of this combination therapy.
局限性硬皮病(LS)的特征是胶原蛋白过度沉积,导致真皮、皮下组织或两者增厚。大多数局限性硬皮病患者的预后与受影响组织的类型和阶段直接相关。未经治疗的患者面临的主要挑战不是死亡风险增加,而是皮肤、肌肉和骨骼异常导致的畸形和生长缺陷。治疗根据病变的类型和阶段进行个体化,可能包括药物治疗和非药物治疗。在药物治疗方法中,甲氨蝶呤与全身性糖皮质激素目前是主要的治疗手段。需要更多的对照试验来确定这种联合治疗的疗程和维持剂量。