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篇名 抗磷脂質症候群
卷期 23:5
並列篇名 Antiphospholipid Syndrome
作者 謝松洲
頁次 324-329
關鍵字 抗磷脂質症候群抗磷脂質抗體反覆性流產動靜脈栓塞免疫療法Antiphospholipid syndromeAntiphsopholipid antibodies, aPLRecurrent pregnancy lossArterial and venous thromboembolismImmunotherapyScopusTSCI
出刊日期 201210

中文摘要

抗磷脂質症候群最早於1983年由英國Graham Hughes 教授所提出。但受限於臨床表現的多樣性及實驗室診斷的不足,在早期這個重要的疾病並沒有受到太多的重視。而由於造成臨床表現最重要的致病機轉來自於動靜脈栓塞,因此早期以抗血栓為主要的療法,雖然緩解了部分病患的症狀或疾病活性,但是仍然有相當的病患無法獲得良好的改善。近年來隨著對各種臨床表現的認知大大提高了醫師們的警覺性,而檢驗方法的敏感性及特異性的改進及標準化,更有助於對診斷的佐證,同時由於對致病機轉的研究進展逐步導入自體免疫的治療觀念,使用各種免疫調節藥物。更對於頑固性或急性惡化的病情導入化學藥物的療法,血漿置換或免疫球蛋白的治療等。抗磷脂質症候群幾乎涵蓋所有身體組織器官可能的病變或功能障礙,例如神經的病變尤其是中風、肺動脈高壓、皮膚的網狀青斑、部分的血小板減少症及反覆性流產等。近年來由於診斷學的進步,加上積極的介入治療已大大改變了這些疾病的預後,不過最重要的還是臨床上對各種早期徵候的警覺性。

英文摘要

Antiphospholipid syndrome was first described by Dr. Hughes in 1983 by clinical observation and communication for a long time. Because of limitations by markedly various clinical manifestations and unavailable reliable laboratory tests, antiphospholipid syndrome is underdiagnosis until now. The major events of ischemia and tissue damage comes from the mechanisms of thromboembolism. Therefore, some patients are benefit from the introduction of anticoagulant therapy, but this kind therapy is still not enough for other patients, especially in the refractory or serious cases. The basic pathogenesis of antiphospholipid syndrome is autoimmunity, immunotherapy with disease-modified antirheumatic drugs are introduced for patients not well response to anticoagulant therapy. These therapies also include plasma exchange, immunoglobulin therapy and cytotoxic agent therapy etc., now for severe or refractory cases, such as catastrophic antiphospholipid syndrome. The clinical manifestations involve nearly every organ system, such as stroke in neurology, cardiac events, pulmonary hypertension in cardiology, livedo reticularis in dermatology and recurrent fetal loss in obstetric field etc. Although there are much progress in the diagnosis and treatment of antiphospholipid syndrome in recent years, including more sensitive and standardized laboratory tests helpful for the confirmation of diagnosis, some patients are still not benefit from the understanding of antiphospholipid syndrome. Therefore, these various clinical manifestations are the most important clues to further classify the possibility of antiphospholipid syndrome. But highly suspicious of antiphospholipid syndrome is most crucial to the diagnosis of this underestimated disease.

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