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抗磷脂综合征与妊娠。

Antiphospholipid syndrome and pregnancy.

机构信息

Unit of Surgical, Microsurgical and Medical Sciences, Department of Dermatology, University of Sassari, Sassari, Italy -

Unit of Surgical, Microsurgical and Medical Sciences, Department of Dermatology, University of Sassari, Sassari, Italy.

出版信息

G Ital Dermatol Venereol. 2019 Jun;154(3):277-285. doi: 10.23736/S0392-0488.18.06152-7. Epub 2018 Oct 29.

Abstract

Antiphospholipid syndrome (APS) is an autoimmune thrombophilic condition characterized by the onset of venous and/or arterial thrombosis, often multiple, and pregnancy morbidity in a background of antiphospholipid antibodies (aPL) positivity. Some patients can be carrier of aPL with no clinical symptoms, in other cases clinical manifestation can range from the classical presentation to an acute life-threatening condition named catastrophic APS. APS can be considered as primary or associated to other disease, however pregnancy acts as a triggering factor on a susceptible background that lead to the clinical manifestations through immunological and non-immunological mechanism. APS is characterized by thrombotic manifestation involving both venous and arterial vessels of different size, in any part of the body the clinical presentation can be extremely polymorphic. Livedo reticularis is the most common cutaneous finding, however it is not specific for APS and other cutaneous manifestation such as ulcers or purpura can be the only presentation of APS. Diagnosis is established by clinical and laboratory criteria. Counselling, pregnancy plan and monitoring during pregnancy is mandatory in APS patients. Treatment can be challenging, and it is mainly based on the use of anticoagulant and antiaggregant medications. A literature review on PubMed about APS and pregnancy was performed, and the significant information in conjunction with our clinical experience lead to the drafting of this paper. We present a review of APS and its management in pregnancy, with a special focus on the dermatological presentation and the role of the dermatologists in diagnosing and managing this potential life-threatening condition.

摘要

抗磷脂综合征(APS)是一种自身免疫性血栓形成性疾病,其特征是在抗磷脂抗体(aPL)阳性的背景下出现静脉和/或动脉血栓形成,通常为多发性,以及妊娠并发症。一些患者可能携带 aPL 而没有临床症状,在其他情况下,临床表现范围从经典表现到一种称为灾难性 APS 的急性危及生命的情况。APS 可被认为是原发性的或与其他疾病相关的,但妊娠是在易感背景下作为触发因素,通过免疫和非免疫机制导致临床表现。APS 的特征是血栓形成表现,涉及不同大小的静脉和动脉血管,在身体的任何部位,临床表现都可能极其多样。网状青斑是最常见的皮肤表现,但它不是 APS 的特异性表现,其他皮肤表现如溃疡或紫癜可能是 APS 的唯一表现。诊断是通过临床和实验室标准确定的。APS 患者必须进行咨询、妊娠计划和妊娠期间的监测。治疗可能具有挑战性,主要基于抗凝和抗血小板药物的使用。我们在 PubMed 上进行了关于 APS 和妊娠的文献综述,并结合我们的临床经验,得出了这篇论文的要点。我们回顾了 APS 及其在妊娠中的管理,特别关注皮肤科表现以及皮肤科医生在诊断和管理这种潜在危及生命的疾病中的作用。

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