硬皮症(scleroderma) 或稱全身性硬化症(systemic sclerosis; SSc) 是以微血管病變為主的 自體免疫疾病。免疫的失調、纖維母細胞的功能異常導致自體免疫抗體形成、血管壁異常增 生、組織缺血以及細胞外基質增生沉積，持續的免疫系統活化發炎導致皮膚與內臟器官的纖 維化與失能。典型的臨床表現有CREST (calcinosis, Raynaud’s phenomenon, esophageal motility dysfunction, sclerodactyly, and telangiectasia)，嚴重導致間質性肺疾病、肺高壓、急性腎衰竭危 機。硬皮症診斷原沿用美國風濕病學會在1980 年所制定的診斷條件，在2013 年由美國風濕 病學會與歐洲抗風濕病聯盟一同制定了新的歸類標準，新標準納入了特定甲褶血管鏡檢查特 異血管表現，除了能提早確診外更提高了硬皮症診斷的專一度與敏感度。治療上除了免疫調 節劑與免疫抑制劑之外，有更多證據顯示肺動脈高壓的治療對患者生存率有絕對影響。本文 將介紹新制硬皮症歸類標準與甲褶血管鏡之應用，並將回顧藥物治療最新建議，以期能提高 硬皮症患者早期診斷率並及早介入治療，減少併發症的產生。

Systemic sclerosis (scleroderma; SSc) is a chronic multisystem autoimmune disease characterized by a vasculopathy which divided into limited cutaneous disease and diffuse cutaneous disease. The clinical manifestations of this disease are diffuse fibrosis of skin and multiple internal organs. A newly diagnosed criterion was established by American College of Rheumatology (ACR) and The European League Against Rheumatism (EULAR) in 2013. Compare to ACR 1980 criteria, it included capillary microscopy evaluation and scleroderma specific autoantibody and provided higher sensitivity and specificity. This review discusses the newly diagnostic criteria, capillary microscopic vascular pattern, newly therapeutic recommendations and developing assessment tools for scleroderma.