綜論全身性紅斑性狼瘡及2019年歐洲抗風濕病聯盟暨美國風濕病醫學會治療指引

Systemic Review of Epidemiology, Pathogenesis, Clinical Manifestations, Classification, and 2019 Updated Recommendation of Managements for Systemic Lupus Erythematosus

黃雅君(Ya-Chun Huang) ； 翁孟玉(Meng-Yu Weng)

《內科學誌》 32卷2期 (2021/04) Pp. 108-120

https://doi.org/10.6314/JIMT.202104_32(2).04

摘要

全身性紅斑性狼瘡乃一好發在育齡期女性的自體免疫疾病，因補體系統的活化及免疫複合體的沉積而導致全身性複雜多變的症狀，包含皮膚黏膜、關節、肋膜肺臟、心血管、胃腸道、腎臟、神經系統及周邊血液等侵犯表現；自體免疫抗體檢查在診斷扮演不可或缺的角色。除了需與其他疾病作詳細鑑別之外，確診仰賴臨床醫師的專業判斷。由於各器官侵犯表現特異，2019年歐洲抗風濕病聯盟暨美國風濕病醫學會提出更新版紅斑性狼瘡治療指引，同年亦提出對狼瘡腎炎及抗磷脂質症候群的治療指引。本文將紅斑性狼瘡之流行病學、病生理機轉、臨床表現、診斷及2019年最新治療建議做一系統性文獻回顧及整理。

關鍵字

全身性紅斑性狼瘡（Systemic lupus erythematosus） ； 皮膚型紅斑性狼瘡（Cutaneous lupus erythematosus） ； 神經精神性狼瘡（Neuropsychiatric systemic lupus erythematosus） ； 狼瘡腎炎（Lupus nephritis） ； 抗磷脂抗體症候群（Antiphospholipid syndrome）

並列摘要

Systemic lupus erythematosus (SLE) is an autoimmune disease that mostly affects women in reproductive age. The complexity of the signs and symptoms of SLE may attribute to the activation of complement system and subsequent immunocomplex deposition in a susceptible subject. Therefore, associated differential diagnosis with other diseases is essential. The clinical classification of SLE remains the challenge for rheumatologists. In 2019, EULAR and ACR published the updated recommendations for management of SLE; the recommendations for the managements of lupus nephritis and antiphospholipid syndrome (APS) were also published last year. In this article, we aim to provide a comprehensive review of epidemiology, pathogenesis, clinical manifestations, classification, and updated recommendations published in 2019 of SLE.