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桥脑梗死作为系统性红斑狼疮中灾难性抗磷脂综合征的首发表现。

Pontine Infarct as Initial Presentation of Catastrophic Antiphospholipid Syndrome in Systemic Lupus Erythematous.

机构信息

Department of Medicine, Universiti Kebangsaan Malaysia (UKM) Medical Centre, Cheras, Kuala Lumpur, Malaysia.

出版信息

Acta Med Indones. 2022 Apr;54(2):288-291.

Abstract

Antiphospholipid syndrome (APLS) is an autoimmune condition which commonly manifests as an arterial or venous thrombosis affecting medium to large vessels, with the presence of antiphospholipid antibodies. APLS can be a primary disease by itself, or secondary to other autoimmune diseases, such as Systemic Lupus Erythematosus (SLE). Catastrophic APLS is a rare but a fatal sequelae of APLS, affecting up to three or more organs, and progresses rapidly with a high mortality rate. We report a case of catastrophic APLS in a young woman with underlying SLE who presented to us with multiple cranial nerve palsies due to bilateral pontine infarct, and eventually developed deep vein thrombosis and pulmonary embolism during the course of the illness. She was treated with high dose corticosteroids and intravenous cyclophosphamide with biochemical improvement. In this case report, we would like to highlight the fact that our patient had bilateral pontine infarcts as the initial presentation, with no inciting events and  antiphospholipid antibodies were negative during the acute illness.

摘要

抗磷脂综合征(APLS)是一种自身免疫性疾病,通常表现为影响中到大血管的动脉或静脉血栓形成,同时存在抗磷脂抗体。APLS 可以是一种原发性疾病,也可以是继发于其他自身免疫性疾病,如系统性红斑狼疮(SLE)。灾难性抗磷脂综合征是 APLS 的一种罕见但致命的后遗症,影响多达三个或更多器官,并且进展迅速,死亡率高。我们报告了一例年轻女性的灾难性 APLS 病例,该患者患有基础 SLE,因双侧脑桥梗死导致多发性颅神经麻痹,最终在疾病过程中发展为深静脉血栓形成和肺栓塞。她接受了大剂量皮质类固醇和静脉环磷酰胺治疗,生化指标有所改善。在这个病例报告中,我们想强调的是,我们的患者最初表现为双侧脑桥梗死,没有诱发事件,并且在急性疾病期间抗磷脂抗体阴性。

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