硬皮症

Scleroderma

硬皮症(scleroderma)主要以微血管病變為主的罕見自體免疫疾病，會使皮膚、血管壁異常增生及器官纖維化，逐漸失去其生活機能。本案例為70歲女性，因呼吸窘迫入院治療，入院後發現其肢體皮膚變厚且硬化，關節僵硬無法彎曲，指頭末端有零星紫色斑塊，進一步與風濕免疫醫師討論，並依照美國風濕病學會與歐洲抗風濕病聯盟制定之分類標準，確立診斷為硬皮症。給予免疫抑制劑及單株抗體治療後，個案皮膚硬化及僵硬程度大幅改善，病情穩定後返家，持續門診追蹤治療。硬皮症的初期在臨床表現上並無特異性，常被忽視而延誤治療，因此早期診斷、早期治療，是可以大幅降低併發症的發生。

Systemic sclerosis (scleroderma) is a rare autoimmune disease characterized by microvascular abnormalities, causing abnormal skin proliferation, blood vessel wall changes, and organ fibrosis, leading to loss of function. We describe a case of a 70-year-old woman admitted for respiratory distress, experiencing rapid lung and kidney function decline requiring intubation and hemodialysis. Physical examination revealed skin thickening, joint stiffness, and purplish patches on the fingertips. After consulting rheumatology experts and following diagnostic criteria, systemic sclerosis was confirmed. Treatment with immunosuppressive agents and monoclonal antibodies improved skin sclerosis and stiffness, reducing complications. Post-treatment, the patient's mobility improved, and she was discharged for outpatient follow-up. Clinical diagnosis of systemic sclerosis is often delayed, underscoring the importance of nurses in assessment, collaboration, and early intervention to mitigate complications.