Department of Medicine II, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Lupus. 2012 Dec;21(14):1506-14. doi: 10.1177/0961203312458469. Epub 2012 Aug 30.
To study the clinical and immunological manifestations of antiphospholipid syndrome (APS) in the Japanese population by a single-centre registration.
In this retrospective cohort study, 141 consecutive patients with APS, fulfilling the Sydney revised Sapporo criteria for definite APS, who visited our autoimmune clinic from 1988 to 2010, were recruited and followed up. All the patients were interviewed and underwent a general physical examination by qualified rheumatologists on the day of blood sampling.
The population comprised 119 woman and 22 men with a mean age at diagnosis of 44 years (range 9-79 years). Seventy patients (49.6%) had primary APS, and 71 (50.4%) had systemic lupus erythematosus. The prevalence of thrombosis was 85.8 per cent, arterial thrombosis was found in 93 patients (66.0%) and venous thrombosis was found in 46 patients (32.6%). The most common thrombosis was cerebral infarction [86/141 (61.0%)] followed by deep vein thrombosis [33/141 (23.4%)]. Among 70 pregnant women, 45 (64.3%) had obstetric complications. Lupus anticoagulant was detected in 116 patients (82.3%), anticardiolipin antibodies in 83 (58.9%), anti-β2 glycoprotein I antibodies in 73 (51.8%) and phosphatidylserine-dependent antiprothrombin antibodies in 98 (69.5%).
High prevalence of arterial thrombosis was noted in Japanese patients with APS. The profile of heterogeneous and complex clinical manifestations was substantiated in Japanese patients with APS.
通过单中心登记研究，探讨日本人群抗磷脂综合征（APS）的临床和免疫学表现。
本回顾性队列研究纳入了 1988 年至 2010 年期间在我们自身免疫诊所就诊的 141 例符合悉尼修订版萨普洛标准的确诊 APS 患者。所有患者均在采血当天由合格的风湿病学家进行访谈和全面体格检查。
患者人群包括 119 名女性和 22 名男性，诊断时的平均年龄为 44 岁（范围 9-79 岁）。70 例（49.6%）为原发性 APS，71 例（50.4%）为系统性红斑狼疮。血栓形成的患病率为 85.8%，93 例（66.0%）存在动脉血栓形成，46 例（32.6%）存在静脉血栓形成。最常见的血栓是脑梗死[86/141（61.0%）]，其次是深静脉血栓形成[33/141（23.4%）]。在 70 例妊娠妇女中，45 例（64.3%）存在产科并发症。116 例（82.3%）患者检测到狼疮抗凝物，83 例（58.9%）抗心磷脂抗体，73 例（51.8%）抗β2糖蛋白 I 抗体，98 例（69.5%）抗磷脂酰丝氨酸依赖的凝血酶原抗体。
日本 APS 患者的动脉血栓形成患病率较高。日本 APS 患者的临床表现呈现出异质性和复杂性。
