以嚴重的心臟瓣膜疾病為全身性紅斑狼瘡的表徵：與抗燐脂質抗體之關係

Severe Valvular Heart Disease associated with Systemic Lupus Erythematosus: Its Relation to Antiphospholipid Antibodies

最近文獻已提出抗燐脂質抗體與全身性紅斑狼瘡併有心臟瓣膜疾病或心內膜炎具有關聯性，但是其發生率尚未有系統性的研究。從已報告的病例獲悉嚴重的瓣膜疾病都是紅斑狼瘡進行一段時間後才被診斷出。我們提出三位全身性紅斑狼瘡併有明顯血行異常的瓣膜閉鎖不全的病患。這三例都有心臟症狀或心衰竭而住院。心臟超音波檢查顯示瓣膜閉鎖不全，瓣膜脫垂或肥厚。三例都具有IgM和IgG抗牛心脂素抗體；其中兩例力價相當高。兩例有血小板減少症。所有病人均無心臟外血栓形成。兩例於紅斑狼瘡被確定後不久即死亡；其中一例死於心衰竭併發敗血症，另一例突然死於循環性虛脫。吾人認為血行嚴重異常的瓣膜閉鎖不全是此三例紅斑狼瘡的表徵，而且與抗燐脂質抗體有關。紅斑狼瘡病人以瓣膜閉鎖不全作為表徵雖少，但卻足以造成心臟瓣膜疾病鑑別診斷上的困擾，而且死亡率也極高。

A total of three patients with systemic lupus erythematosus (SLE) presenting remarkable valve insufficiency was reported. All were hospitalized because of overt congestive heart failure. Mitral regurgitation was echocardiographically demonstrated in association with valvular prolapse and thickening. Most significantly, IgG and IgM anticardiolipin antibodies were found in all three patients, two with high titer. Despite two patients had thrombocytopenia, none had detectable thrombosis. Two succumbed to sepsis and sudden circulatory collapse shortly after the diagnosis of SLE. On the whole, severe valvular insufficiency in assoication with anti-phospholipid antibodies develops as manifestation of SLE, which cause problems in differential diagnosis, and may be ominous in prognosis.