災難性抗磷脂症候群－病例報告與文獻回顧

Catastrophic Antiphospholipid Syndrome

胡瑜峰(Yu-Feng Hu) ； 林逸君(Yi-Chun Lin) ； 梁統華(Toong-Hua Liang) ； 林孝義(Hsiao-Yi Lin)

《內科學誌》 16卷1期 (2005/02) Pp. 33-41

https://doi.org/10.6314/JIMT.2005.16(1).05

摘要

抗磷脂症候群在臨床上並不少見，表現十分多樣性。從典型的動靜脈栓塞、流產或早產、血小板低下，到急性腎衰竭、肺動脈高壓、急性呼吸窘迫症候群、腦中風、癲癇、心肌梗塞、心內膜炎、腎上腺衰竭、神經病變…等均有可能發生。雖然可能有嚴重的併發症，但診斷上往往被忽略、需要具有高度的警覺。抗磷脂症候群可以是原發性的，也可續發於其他疾病，最常見於全身性紅斑性狼瘡。抗磷脂症候群症狀上與紅斑性狼瘡的血管炎表現難以鑑別，而治療上卻南轅北轍，抗磷脂症候群以抗凝血劑為主體，而紅斑性狼瘡則是類固醇。是否能及時診斷並治療、預防栓塞的復發，對病患的預後有重大的影響。在診斷上，需要有動靜脈栓塞或妊娠併發症其中之一，再加上抗磷脂抗體陽性始能確定。
災難性抗磷脂症候群是抗磷脂症候群中罕見的急症，大多有特別的誘發因子，最常見為感染或手術。患者在一星期內有三個以上的器官或組織發生栓塞，病理上以小血管之瀰漫性栓塞為其特點。確定病例必須滿足四項標準：三個以上的器官、系統或組織受到影響；所有表現在一週內發生；至少一個器官或組織的切片證實有小血管瀰漫性栓塞；實驗室檢查確定具有抗磷脂抗體。死亡率高達五成，而死因大多為多重器官衰竭。如能早期發現，去除誘發因子，並以抗凝血劑、類固醇治療，必要時併用血漿置換法或靜脈注射免疫球蛋白，或可改善存活率。本文將以一個災難性抗磷脂症候群的案例為引言，進而介紹抗磷脂症候群之全貌。

關鍵字

抗磷脂症候群 ； 災難性抗磷脂症候群 ； 抗磷脂抗體 ； 磷脂 ； 心磷脂

並列摘要

Antiphospholipid syndrome (APS) is not uncommon and contains a variety of manifestations including arterial or venous thrombosis, abortion or premature labor, thrombocytopenia, acute renal failure, acute respiratory distress syndrome, stroke, seizure, myocardial infarction, neuropathy etc. Because this syndrome may potentially lead to severe complications, it needs clinical suspicion to make the early diagnosis. APS may be divided into several categories. ”Primary” APS occurs in patients without clinical evidence of another autoimmune disease, whereas ”secondary” APS takes place in association with autoimmune or other diseases. Systemic lupus erythematosus is by far the most common disease accompanied by which APS develops. The manifestations of APS need to be differentiated from that of lupus vasculitis for proper treatment. For instance, instead of steroid for vasculitis, anticoagulant is used for APS. Furthermore, it is of great influence on the prognosis to make an early diagnosis and initiate prompt treatment. To diagnose APS, at least one of two clinical criteria (vascular thrombosis and complications of pregnancy) and at least one of two laboratory criteria (lupus antibody and anticardiolipin antibody) must be met. Catastrophic antiphospholipid syndrome (CAPS) is a rare medical emergency. Most of the CAPS are preceded by a precipitating incident, mainly infections or surgeries. Definite CAPS need to meet all four criteria including evidence of involvement of three or more organs, systems and/or tissues development of manifestations simultaneously or in less than a week、confirmation by histopathology of small vessel occlusion in at least one organ or tissue and laboratory confirmation of the presence of antiphospholipid antibodies. The mortality rate is around 50%, and death usually results from multi-organ failure. Early diagnosis, elimination of possible precipitating factors, treatment with anticoagulant and corticosteroids, and plasma exchange or intravenous immunoglobulin if necessary, may survive from the very fatal disease.

並列關鍵字

Antiphospholipid syndrome ； Catastrophic antiphospholipid syndrome ； Antiphospholipid antibody ； Phospholipid ； Cardiolipin