Falcini F, Trapani S, Taccetti G, Tafi L
Dipartimento di Pediatria, Università degli Studi, Firenze, Italia.
Pediatr Med Chir. 1990 Nov-Dec;12(6):593-9.
Systemic sclerosis is a multisystem disease of unknown cause, characterized by inflammation, vascular and fibrotic changes with excessive accumulation of connective tissue. The lesions may involve the skin and various internal organs (kidney, lung, gastrointestinal tract and heart). The first symptom is usually Raynaud's phenomenon, followed by skin changes; at the beginning the skin is swollen and oedematous, and then becomes thick, taut, shiny and atrophic. The prognosis of SS depends mainly on the severity of visceral involvement. The treatment of SS consists of drugs that improve the microcirculation and reduce collagen proliferation, such as calcium-antagonists (nicardipine) and D-Penicillamine.
系统性硬化症是一种病因不明的多系统疾病，其特征为炎症、血管及纤维化改变，伴有结缔组织过度积聚。病变可能累及皮肤和各种内脏器官（肾脏、肺、胃肠道和心脏）。首发症状通常为雷诺现象，随后出现皮肤改变；起初皮肤肿胀、水肿，之后变得增厚、紧绷、发亮且萎缩。系统性硬化症的预后主要取决于内脏受累的严重程度。系统性硬化症的治疗包括改善微循环和减少胶原增生的药物，如钙拮抗剂（尼卡地平）和青霉胺。
