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抗磷脂综合征合并狼疮性心肌炎患者出现非出血性双侧肾上腺梗死。

Non-haemorrhagic, bilateral adrenal infarction in a patient with antiphospholipid syndrome along with lupus myocarditis.

作者信息

Batt Nicholas Marinus, Malik Dean, Harvie Miranda, Sheth Hemant

机构信息

Department of Surgery, London North West Healthcare Trust, Ealing, UK.

Department of Radiology, London North West Healthcare Trust, Ealing, UK.

出版信息

BMJ Case Rep. 2016 Jul 20;2016:bcr2016216364. doi: 10.1136/bcr-2016-216364.

Abstract

A 40-year-old woman with antiphospholipid syndrome presented with a 5-day history of right upper quadrant (RUQ) pain, radiating posteriorly, associated with fever and vomiting. She was admitted 1-week prior with an upper respiratory infection and erythema multiforme. Clinical assessment revealed sepsis with RUQ tenderness and positive Murphy's sign. Laboratory results showed raised inflammatory markers, along with renal and liver impairment. CT showed bilateral adrenal infarction and inferior vena cava thrombus. The patient was managed for sepsis and started on heparin. Further immunological investigations revealed a diagnosis of systemic lupus erythematous, an exacerbation of which culminated in lupus myocarditis. This case illustrates the importance of promptly recognising adrenal insufficiency in patients with antiphospholipid syndrome and the possible causative agents, which require careful consideration and exclusion to prevent further thrombotic events. It also highlights the importance of undertaking imaging, namely CT, in patients with antiphospholipid syndrome presenting with abdominal pain as well as considering concomitant autoimmune conditions.

摘要

一名患有抗磷脂综合征的40岁女性,出现右上腹(RUQ)疼痛5天,疼痛向后放射,伴有发热和呕吐。她在1周前因上呼吸道感染和多形红斑入院。临床评估显示为败血症,伴有右上腹压痛和墨菲氏征阳性。实验室检查结果显示炎症标志物升高,同时伴有肾和肝功能损害。CT显示双侧肾上腺梗死和下腔静脉血栓形成。该患者接受了败血症治疗,并开始使用肝素。进一步的免疫学检查确诊为系统性红斑狼疮,病情加重最终导致狼疮性心肌炎。该病例说明了在抗磷脂综合征患者中及时识别肾上腺功能不全的重要性以及可能的致病因素,需要仔细考虑和排除这些因素以防止进一步的血栓形成事件。它还强调了对出现腹痛的抗磷脂综合征患者进行影像学检查(即CT)以及考虑合并自身免疫性疾病的重要性。

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Guidelines on the investigation and management of antiphospholipid syndrome.抗磷脂综合征的调查与管理指南
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