Cranley James, Krishnan Unni, Tweed Katharine, Duehmke Rudolf Martin
Cardiology, Royal Papworth Hospital NHS Foundation Trust, Cambridge, UK.
Cardiology, Addenbrooke's Hospital NHS Foundation Trust, Cambridge, UK.
BMJ Case Rep. 2019 Jan 10;12(1):bcr-2018-225495. doi: 10.1136/bcr-2018-225495.
A 51-year-old woman with known primary antiphospholipid syndrome presented with a 4-day history of chest and abdominal pain, inferior ST-segment elevation on a 12-lead ECG and a subtherapeutic international normalised ratio. In view of a significantly raised high-sensitivity troponin I assay, inferior wall hypokinesis on transthoracic echocardiography and despite unobstructed epicardial vessels on emergency coronary angiography, a diagnosis of myocardial infarction was made. Furthermore, the patient also developed both bilateral adrenal haemorrhages leading to acute adrenal insufficiency and microvascular thrombotic renal disease concurrently. The patient therefore fulfilled the diagnostic criteria for catastrophic antiphospholipid syndrome presenting with cardiac, endocrine and renal involvement. Early diagnosis permitted appropriate treatment with anticoagulation, dual antiplatelet therapy, secondary prevention and corticosteroid replacement therapy and led to a full recovery. This case highlights first the importance of adequate anticoagulation in antiphospholipid syndrome and, second, the potentially fatal, multiorgan complication of failure to do so.
一名患有原发性抗磷脂综合征的51岁女性，出现胸痛和腹痛4天，12导联心电图显示下壁ST段抬高，国际标准化比值低于治疗水平。鉴于高敏肌钙蛋白I检测显著升高，经胸超声心动图显示下壁运动减弱，尽管急诊冠状动脉造影显示心外膜血管无阻塞，但仍诊断为心肌梗死。此外，患者同时还出现双侧肾上腺出血，导致急性肾上腺功能不全和微血管血栓性肾病。因此，该患者符合灾难性抗磷脂综合征的诊断标准，伴有心脏、内分泌和肾脏受累。早期诊断允许进行适当的抗凝、双联抗血小板治疗、二级预防和皮质类固醇替代治疗，最终实现了完全康复。该病例首先强调了抗磷脂综合征中充分抗凝的重要性，其次强调了未能充分抗凝可能导致的致命性多器官并发症。
