抗磷脂综合征的心脏病变

目的分析抗磷脂综合征合并心脏受累的临床特点、治疗和预后,提高对本病的认识。方法对72例抗磷脂综合征患者临床资料进行回顾性分析。结果72例抗磷脂综合征患者中,心脏受累患者48例,二尖瓣受累发病率最高。心脏受累与血栓事件显著相关,与部分激活的凝血活酶时间延长相关。瓣膜病变与脑梗死和器官血栓事件相关。20例有血栓事件并心脏受累的抗磷脂综合征患者接受了抗凝治疗,未再发生血栓事件。结论抗磷脂综合征累及心脏表现为瓣膜异常、冠状动脉血栓、心肌受累和肺动脉高压等,心脏受累可能与高凝状态相关。     

抗磷脂综合征的心血管系统表现

抗磷脂综合征（APS）是一种累及多器官的系统性自身免疫性疾病,临床以反复发作的动静脉血栓形成、自发性流产、血小板减少以及持续的血清抗磷脂抗体阳性为主要特征。心脏是APS的重要靶器官之一。本文从APS的心血管系统表现如心瓣膜病变、心肌梗死、心腔内血栓形成、冠状动脉微血管血栓形成及治疗策略方面作一综述。     

Treatment of the antiphospholipid antibody syndrome

Numerous questions exist regarding the proper management of patients with the antiphospholipid antibody syndrome (APS). Several recent, randomized-controlled trials have been conducted that attempt to answer the fundamental questions of whom to treat, how to treat, and for how long treatment should last. These studies suggest that APS should be categorized by disease manifestations, and these presentations may have different treatment algorithms. A better understanding of the immune mechanisms that govern thrombosis in this syndrome has led to a wide array of innovative treatment approaches that will require meticulous study.     

Antiphospholipid antibody and antiphospholipid antibody syndrome.

Over the past year, many reports have been published on a variety of clinical manifestations related to antiphospholipid antibodies. The low prevalence of anticardiolipin antibodies with the rare occurrence of thrombosis and a low rate of fetal loss in studies in Malaysia and China showed a potential role for local factors. A study of cross-reactive idiotype of the anticardiolipin antibody suggested that anticardiolipin antibodies are derived from a set of natural autoreactive clones. Regarding the pathogenic role of the antiphospholipid antibody, evidence has been presented that the epitopes formed between cardiolipin and beta 2 glycoprotein I are the targets of the antiphospholipid antibody. Complement activation, abnormalities of natural anticoagulants such as protein S deficiency, and genetic association with DR4, DR7, and DRw53 have also been studied.     

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.     

Progression of antiphospholipid antibody syndrome to catastrophic antiphospholipid antibody syndrome acutely with cessation of antithrombotic therapy

Catastrophic antiphospholipid antibody syndrome (CAPS) is a serious condition that is often unrecognised with a high mortality. Cessation of anticoagulation in antiphospholipid antibody syndrome (APS) can have devastating consequences with progression to CAPS. Making a diagnosis of APS can however be challenging because of the evolving diagnostic criteria and difficulty in confirming thromboses. Management of these patients can also be complex, especially in those with coexistent thrombocytopenia. New potential treatments are emerging targeted on the immunomodulation of APS rather than just prevention of thrombosis. This article aims to highlight these diagnostic and management difficulties by reporting and discussing three cases of APS with progression to CAPS following cessation of anticoagulation, one with fatal consequences, with confirmation of CAPS on autopsy, and two with successful treatment and outcomes.     

Thrombotic microangiopathy and the antiphospholipid antibody syndrome.

A 23-year-old woman with the antiphospholipid syndrome developed severe thrombocytopenia (14 x 10(9)/l) and microangiopathic hemolytic anemia after plasma exchange. A relation was suspected between rising anticardiolipin level and development of thrombotic microangiopathy. The mechanism responsible may be interference between anticardiolipin antibody, platelet membrane and endothelial cell.     

Management of thrombosis in antiphospholipid antibody syndrome

Antiphospholipid antibody positive patients are at risk for venous and arterial thrombosis. The risk of recurrent thromboembolism is high. Although the standard of care is high-intensity warfarin after a thromboembolic event, some studies indicate that this degree of anticoagulation is not needed. There is an urgent need of clinical trials to address management of thrombosis in antiphospholipid syndrome.     

Cardiovascular abnormalities in the marfanoid hypermobility syndrome

Two patients are described who had the body habitus of the Marfan syndrome, cutaneous hyperextensibility, and atrophic “cigarette paper” scars. One had mild joint hypermobility and the other had generalized ligamentous laxity. Both individuals are considered to be examples of the Marfanoid hypermobility syndrome, a generalized heritable connective tissue disease with features of, but genetically distinct from, the Marfan and Ehlers-Danlos syndromes. One patient had a “floppy” mitrai valve and the other had an aortic aneurysm due to cystic medionecrosis of the aorta; both findings provide evidence that cardiovascular disease may be a significant feature of this syndrome.     

Silent myocardial infarction and antiphospholipid antibody syndrome

We describe a case of 18-year-old woman followed for 3 years for systemic lupus erythematosis (SLE) complicated of a nephrectomy. Having like only factor of cardiovascular risk a balanced arterial hypertension. The patient was hospitalized because of choreo-athetosic's movement. We discovered fortuitously during this hospitalization an inferior myocardial necrosis as well as a mitral regurgitation. Coronary angiography was normal and the ventriculography showed an akinesy in the inferior territory. Biology made it possible to pose the diagnosis of antiphospholipid antibody syndrome (APS) on (SLE). We suppose that surgery started myocardial necrosis and underline through this case interest of early identification and appropriate treatment of APS as well as a narrow monitoring particularly in young patients candidates to surgery.     

Evidence-based management of thrombosis in the antiphospholipid antibody syndrome

Recommendations for the management of thrombosis in the antiphospholipid antibody syndrome have been based largely on retrospective case series. Several clinical trials have been recently published on the management of thrombosis in general. Two clinical trials have specifically addressed thrombosis in antiphospholipid antibody syndrome, and one is underway. These new clinical trials have challenged the previous dogma of a target International Normalized Ratio of 3 to 4 (“high-intensity” warfarin).     

HIV infection and antiphospholipid antibody: literature review and link to the antiphospholipid syndrome

There is a high incidence of antiphospholipid antibodies, detected by assays for anticardiolipin or lupus-like anticoagulant, in HIV disease. However, a link to the antiphospholipid syndrome, with clinical thrombosis, is tenuous. We report a case of a 25-year-old man with undetermined risk factors for HIV presenting with possible antiphospholipid syndrome manifesting as necrotic skin lesions as the initial clinical presentation for HIV. We also review the literature exploring the association between HIV and antiphospholipid syndrome.