Department of Medicine, Institute of Medical Sciences, Banaras Hindu University, Vanarasi, U.P., India; Division of Rheumatology, Institute of Medical Sciences, Banaras Hindu University, Vanarasi, U.P., India.
Int J Rheum Dis. 2013 Oct;16(5):547-55. doi: 10.1111/1756-185X.12150.
To study the clinical and immunological features of primary antiphospholipid syndrome (APS), and to analyze the differences between primary APS and APS associated with autoimmune rheumatic disease (ARD/APS).
This prospective, longitudinal study, carried out from December 2004 to July 2011 included 179 patients with primary APS and 52 patients of ARD/APS diagnosed as per modified 2006 Sapporo's Criteria.
Out of 179 patients of primary APS, 12 were male and 167 were female. The mean age at the time of study entry was 27 ± 4.33 years. Venous thrombosis was noted in 33 (18.43%) patients. Seventeen patients had deep vein thrombosis and 11 (7.19%) had cortical vein and/or cortical sinus thrombosis. Arterial thrombosis was noted in 19 (10.61%) patients, out of which nine had intracranial arterial thrombosis. Thirty-two (17.85%) had recurrent early fetal losses (< 10 weeks) and 97 (54.18%) had late fetal loss (> 10 weeks). Immunoglobulin G (IgG) and IgM aCLA were present in 141 (78.77%) and 32 (17.87%) patients respectively, whereas lupus anticoagulant was present in 99 (55.3%) patients. In patients with bad obstetric outcome, lupus anticoagulant positivity was significantly more prevalent (P < 0.05) than aCLA positivity. Both venous and arterial thrombosis were significantly more common (P < 0.05) in ARD/APS. However, late fetal loss was significantly more prevalent (P < 0.001) in primary APS.
Primary APS may lead to a variety of clinical manifestations due to venous and/or arterial thrombosis, which at times may be lethal. It is also an important cause of early and late pregnancy loss(es) and other pregnancy morbidities.
研究原发性抗磷脂综合征(APS)的临床和免疫学特征,并分析原发性 APS 与伴发自身免疫性风湿病(ARD/APS)的 APS 之间的差异。
本前瞻性、纵向研究于 2004 年 12 月至 2011 年 7 月进行,共纳入 179 例原发性 APS 患者和 52 例符合 2006 年札幌修订标准的 ARD/APS 患者。
179 例原发性 APS 患者中,男性 12 例,女性 167 例,研究入组时的平均年龄为 27±4.33 岁。33 例(18.43%)患者存在静脉血栓形成,17 例为深静脉血栓形成,11 例(7.19%)为皮质静脉和/或皮质窦血栓形成。19 例(10.61%)患者存在动脉血栓形成,其中 9 例为颅内动脉血栓形成。32 例(17.85%)患者发生早期复发性胎儿丢失(<10 周),97 例(54.18%)发生晚期胎儿丢失(>10 周)。141 例(78.77%)和 32 例(17.87%)患者存在 IgG 和 IgM 抗心磷脂抗体,99 例(55.3%)患者存在狼疮抗凝物。不良妊娠结局患者中狼疮抗凝物阳性率显著高于抗心磷脂抗体阳性率(P<0.05)。ARD/APS 患者的静脉和动脉血栓形成更为常见(P<0.05),但原发性 APS 患者的晚期胎儿丢失更为常见(P<0.001)。
原发性 APS 可因静脉和/或动脉血栓形成导致多种临床表现,有时甚至可危及生命,也是早期和晚期妊娠丢失及其他妊娠并发症的重要原因。