抗磷脂综合征174例临床特点及与欧洲数据的比较分析

目的 分析中国人群抗磷脂综合征(AIDS)的临床特点,以提高对这一疾病的认识.方法 回顾性分析1996-2009年在上海仁济医院诊治的APS患者的临床和实验室特点.采用χ2检验.结果 确诊APS 174例,男:女为1:6.6.原发性APS 31例,继发性APS 143例,138例继发于系统性红斑狼疮.141例患者发生血栓事件,以脑梗死、深静脉血栓、肺栓塞为主.62例(44.0%)多部位(≥2处)血栓,45例(31.9%)血栓复发(≥2次),且原发性APS较继发性APS更易复发血栓(P<0.05). 63例发生异常妊娠.7例病理诊断APS肾病(APSN),以肾小球微血栓为特点.174例AlaS中,抗心磷脂抗体阳性112例(64.4%),抗β2糖蛋白Ⅰ抗体阳性103例(59.1%),狼疮抗凝物(LA)阳性50例(28.7%).结论 APS以血栓事件和异常妊娠为主要表现,本组APS主要血栓事件与欧洲数据组一致;原发性APS更易复发血栓事件;肾小球微血栓为APSN常见病理表现之一;同时进行3种抗磷脂抗体检测有助于临床诊断APS.     

抗磷脂综合征165例临床特征与分型

目的 分析抗磷脂综合征(APS)的临床特征与分型.方法 根据2006年更新的APS分类诊断标准及新的临床亚型的定义,回顾性分析北京协和医院住院的165例APS患者的临床分型,总结患者的临床表现及治疗,并分析抗磷脂抗体与血栓的相关性.结果 165例患者中,男:女为1:3.不同临床亚型的分类包括确诊APS 116例(70.3%),可能APS 34例(20.6%),血清阴性APS 10例(6.1%),微血管性APS 5例(3.0%).124例(75.2%)合并其他疾病,其中113例(91.1%)合并自身免疫病,以系统性红斑狼疮常见(79.6%).合并血栓者121例(73.3%),其中静脉血栓68例(56.2%),以下肢深静脉血栓最常见.仅有狼疮抗凝物(LA)阳性和仅有抗心磷脂抗体(ACL)阳性患者的血栓发生率分别为86.0%和65.5%(P<0.05).61例APTT延长的患者中,LA阳性50例(82.0%),ACL阳性34例(55.7%).结论 根据APS临床表现可分为多种临床亚型.APS合并血栓以静脉血栓多见.LA阳性较ACL阳性的患者血栓发生率高.APTT延长与LA的相关性较强.     

抗磷脂综合征相关心血管疾病的表现及新进展

抗磷脂综合征（APS）是以反复发生血栓事件和妊娠并发症以及抗磷脂抗体持续阳性为特点的一种系统性自身免疫性疾病。APS可以影响包括心血管系统在内的多个器官和系统,APS相关心血管疾病会给患者生命造成严重威胁。在2023年更新的美国风湿病学会（ACR）联合欧洲抗风湿病联盟（EULAR）APS分类标准中,将瓣膜病变纳入了APS的临床标准,这也提示应该重视APS合并的心血管疾病。本文将对APS合并的常见心血管疾病的表现及机制进行综述。     

以反复肺动脉栓塞为首发症状的原发性抗磷脂综合征1例

正抗磷脂综合征(antiphospholipid syndrome,APS)是一种自身免疫状态,可以针对某些磷脂结合的血浆蛋白产生自身抗体,与血栓形成(包括静脉和动脉)、妊娠丢失等疾病发生风险相关。发生在没有其他自身免疫性疾病的情况下,称为原发性APS(primary APS,PAPS)。发生在系统性红斑狼疮或其他自身免疫性疾病患者的APS被称为继发性APS(secondary APS,SAPS)。APS特征是血液循环中抗磷脂抗体(antiphospholipid antibodies,APL)持续阳性,APL常用的临床实验室检测是狼     

抗磷脂综合征治疗现状

正抗磷脂综合征(antiphospholipid syndrome,APS)是一种累及全身多系统、器官和组织的复杂的疾病。主要临床表现为血栓事件及产科事件,与抗磷脂抗体(antiphospholipid antibodies,aPL)介导的血栓和炎症机制相关。根据目前流行病学数据提示,APS的人口患病率为每100,000例50例,年发病率为每100,000例2. 1例[1]。APS可以累及全身任何组织器官的血管,包括动脉、静脉及微血管,其中下肢深静脉和大脑动脉是最常见的血栓形成部位[2,3]。APS引起的产科并发症目前也得到了很好的认识,主要临床表现包括子痫或严重子痫前期,妊娠10周前的早产以及中晚期的胎儿死亡[2,4]。除了血栓形成和妊娠并发症外,其他临床特征如持续性血小板减少、溶血性贫血、网状青紫、APS肾病和认知功     

抗膜联蛋白A2抗体在抗磷脂综合征中的作用

目的 研究抗膜联蛋白A2抗体在抗磷脂综合征(APS)、系统性红斑狼疮(SLE)的血栓/病态妊娠中的可能作用.方法 先用分子克隆方法表达纯化出重组膜联蛋白A2,然后以重组膜联蛋白A2为抗原,采用酶联免疫吸附试验(ELISA)法分别检测了,101例APS患者,41例SLE合并血栓患者,124例无血栓的SLE患者及120名健康人的血清中IgG型抗膜联蛋白A2抗体水平.结果 APS组和SLE合并血栓组的IgC型抗膜联蛋白A2抗体阳性率分别为21.8%,26.8%,均品著高于单纯SLE组(6.5%)(P值均<0.0.).IgG型抗膜联蛋白A2抗体与血栓/病态妊娠有关联(P<0.01).IgG型抗膜联蛋白A2抗体对血栓/病态妊娠诊断的敏感性、特异性、预测值分别为0.232、0.935、0.805.结论 IgG型抗膜联蛋白A2抗体与APS和SLE患者的血栓/病态妊娠表现相关,将有助于一些潜在的APS患者的诊断.     

抗磷脂综合征血栓形成的机制

抗磷脂综合征（antiphospholipid syndrome，APS）于1983年由Graham Hughes首次提出，故又称Hughes综合征，是一种自身免疫性疾病，临床表现为反复发生的动、静脉血栓，习惯性流产和血小板减少等，同时有抗磷脂抗体存在（主要为狼疮抗凝物质和抗心磷脂抗体）的实验室证据。分为原发性和继发性，前者无任何病因，后者可继发于自身免疫性疾病，肿瘤，感染，药物等。血栓形成是APS的主要病理基础，也是导致患者死亡的主要原因。研究发现，抗磷脂抗体可见于多种免疫介导的血栓性疾病，提示其对于血栓形成具有关键性的作用。     

纤维蛋白溶解系统异常与抗磷脂综合征

抗磷脂综合征(antiphospholipid syndrome,APS)是由抗磷脂抗体引起的一组临床征象的总称,主要表现为血栓形成、习惯性流产、血小板减少等症状,这些症状可单一出现或多个共同存在。APS患者血清中存在高滴度的抗磷脂抗体(an- tiphospholipid antibody,aPL)。     

临床病例评析——突发胸痛左下肢肿痛颧部红斑

目的:提高临床对APS与SLE之间关系的认识。方法:报道1例以原发APS为首发临床表现,随诊过程中新发皮肤损害、抗dsDNA抗体阳性,转为患者的临床特点及诊治经过,并进行分析讨论。结果:15岁女性患者,病程初期表现为急性肺栓塞、下肢深静脉血栓,抗磷脂抗体高滴度阳性,其他自身抗体阴性,诊断原发性APS,予溶栓抗凝治疗后好...     

抗磷脂抗体综合征血栓形成的机制

抗磷脂抗体综合征 (APS)大多数症状与血栓形成有关 ,其血栓的发病机制目前尚不十分清楚 ,实验和临床结果表明 ,血清中高滴度的抗磷脂抗体 (APA)存在是血栓形成的关键 ,促血凝机理可能与以下几个方面有关。1　 APA对血管内皮细胞和血小板功能的影响细胞膜的主要结构成分是带阴电荷的磷脂 ,在 APS患者中 ,APA损伤血小板和内皮细胞膜 ,导致细胞膜内层带负电荷磷脂暴露于胞膜表面 ,APA与其结合后 ,使内皮细胞功能受损 ,APA一方面使磷脂酶 A2 诱导的花生四烯酸释放减少 ,导致前列环素 (PGI2 )合成降低 ,另一方面可选择性抑制凝血酶介导…     

Pediatric antiphospholipid antibodies and antiphospholipid syndrome

Antiphospholipid syndrome (APS) can occur in children, like adults, with the same diverse spectrum of thrombotic sites but predominately with deep vein thrombosis and stroke. In contrast with adults, however, transient nonthrombogenic antiphospholipid (aPL) antibodies are seen more commonly, usually after childhood infections. In those with "true" aPL antibodies, recurrent thrombotic events seem less frequent than in adults, perhaps reflecting the less prothrombotic hemostatic state of childhood. Children with thrombotic events in APS present difficult management problems, as there is little evidence-based medicine. The duration and intensity of anticoagulation are unresolved management issues, but a target international normalized ratio of 2 to 3 is used by most. Multicenter randomized controlled trials would provide answers to some of these issues but are difficult to organize due to ethical issues and the rarity of the condition. A pediatric APS registry such as the Ped-APS Register is more easy to organize and can yield informative data.     

Pathogenetic mechanisms of antiphospholipid antibody production in antiphospholipid syndrome

Antiphospholiipid syndrome (APS) is an autoimmune disease characterized by the pathological action of antiphospholipid antibodies (aPL), that leads to recurrent pregnancy loss and thrombosis. Despite limited evidence, it is clear that there are both inherited and acquired components of the ontogeny of these antibodies. Animal genetic studies and human familial and population studies highlight the influence of genetic factors in APS, particularly human leukocyte antigen associations. Similarly, both animal and human studies have reported the importance of acquired factors in APS development and infectious agents in particular have a great impact on aPL production. Bacterial and viral agents have been implicated in the induction of autoimmune responses by various mechanisms including molecular mimicry, cryptic autoantigens exposure and apoptosis. In this review we highlight the latest updates with regards to inherited and acquired factors leading to the manufacturing of pathogenic antibodies and APS.     

Managing antiphospholipid antibodies and antiphospholipid syndrome in children

Antiphospholipid antibodies (aPL) and antiphospholipid syndrome (APS) are increasingly being recognized in children. Transient non-pathogenic aPL are often seen after childhood infections, while thrombotic events seem rare in those with true aPL. We discuss the main scenarios faced when dealing with children with aPL--asymtomatic aPL, primary APS and secondary APS. Children with thrombotic events present difficult management problems, as there is little evidence-based medicine in this area. We discuss the manifestations and management of childhood aPL--asymptomatic aPL, primary and secondary APS elucidated with case histories. Insufficient safety data on anticoagulation and limited information on the effects of warfarin, use of aspirin, duration and intensity of anticoagulation are some of the unresolved issues in managing aPL and APS in children. Multicenter randomized controlled trials may provide answers to some of these issues.     

Management of antiphospholipid syndrome

Clinical Rheumatology - Antiphospholipid syndrome (APS) is an autoimmune disease characterised by vascular thrombosis and/or pregnancy morbidity in the presence of persistently positive serum tests...     

Catastrophic antiphospholipid syndrome

Antiphospholipid syndrome (APS) is a thrombotic disorder associated with autoantibodies that target membrane phospholipids and phospholipid-binding proteins, which regulate coagulation. APS is usually characterized by major arterial or venous occlusions, pregnancy complications, or both. In 1992, Asherson described an unusual variant of APS termed the catastrophic antiphospholipid syndrome (also known as Asherson's syndrome), the hallmark of which is rapid multiorgan failure caused by widespread small-vessel thrombi. Empiric treatments have improved the prognosis of patients, but half still die from thrombotic diathesis, even though those who survive the acute stages frequently remain well. Given the persistently high mortality rate, efforts have been underway to facilitate early diagnosis, institute effective treatments in a timely manner and to better understand the cause (or causes) of this extreme condition in order to improve outcomes.     

Catastrophic antiphospholipid syndrome

Catastrophic antiphospholipid syndrome, defined and documented in 1992, is a potentially fatal complication seen in patients with antiphospholipid antibody. It may arise de novo in patients not previously suspected as having an antiphospholipid syndrome, or it may complicate the course of patients currently treated for this syndrome. Precipitating or “trigger” factors have been identified in 55% of patients; the most common of these factors is infection. The precipitating factors should be avoided or energetically treated in patients with the antiphospholipid syndrome in order to prevent this “catastrophic” course. The clinical manifestations are those of multiorgan failure, and unusual vessels or organs can be involved. Treatment of the condition, with emphasis on effective anticoagulation, intravenous steroids, intravenous immunoglobulin, or plasma exchange, should be aggressive to achieve a satisfactory outcome. Regrettably, despite all available therapeutic options at this time, the mortality is still high (greater than 50%).     

Diagnosis of antiphospholipid syndrome

Antiphospholipid syndrome is a multisystem autoimmune disease, characterized by recurrent vascular thrombosis and/or pregnancy losses in the presence of persistently positive antiphospholipid antibodies. In clinical practice, testing for anticardiolipin antibodies and lupus anticoagulant is mandatory for the laboratory diagnosis of antiphospholipid syndrome. Identification of patients with antiphospholipid syndrome is important, as prophylactic anticoagulant therapy may prevent thrombosis from recurring, and treatment during pregnancy can improve fetal and maternal outcome.