Mittal Nitish, Abohelwa Mostafa, Rahman M Rubayat, Shurmur Scott
Texas Tech University Health Science Center, School of Medicine, Class 2022, Lubbock, TX, USA.
Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, TX, USA.
Eur Heart J Case Rep. 2022 May 11;6(5):ytac199. doi: 10.1093/ehjcr/ytac199. eCollection 2022 May.
Antiphospholipid syndrome (APS) is an autoimmune response characterized clinically by arterial or venous thrombosis. One of the rare and series forms of APS is the catastrophic APS (CAPS). The incidence of CAPS has been reported in 0.8% of patients with APS. There have been very few case reports with cardiac involvement in CAPS. Common cardiac manifestations include valvular thickening and lesions, coronary artery disease, and myocardial infarction due to microvascular thrombosis. Here, we are reporting a case of CAPS associated with heart failure and a literature review of similar cases.
A 24-year-old woman with a history of APS presented with shortness of breath and right-sided pleuritic chest pain. Computed tomography pulmonary angiogram revealed new pulmonary emboli in the right lung. After 5 days, she developed high-grade fever with negative infectious workup, acute hypoxic respiratory failure with pulmonary oedema, shock, acute kidney injury, and transthoracic echocardiography showed reduced ejection fraction and global hypokinesia. The constellation of multi-organ failure, symptoms within a week, the presence of antiphospholipid antibodies, and exclusion of other causes, CAPS was diagnosed. The patient showed significant improvement with pulse steroids, IV plasmapheresis and got discharged on oral prednisone taper and anticoagulation with home health.
There are different cardiac complications associated with CAPS, including congestive heart failure, acute coronary syndrome, valvular lesions, and thrombus. Heart failure management in CAPS includes triple therapy of intravenous immune globulin, IV plasmapheresis, and corticosteroids rather than conventional treatment.
抗磷脂综合征（APS）是一种自身免疫反应，临床上以动脉或静脉血栓形成为特征。灾难性抗磷脂综合征（CAPS）是APS罕见的一种严重形式。据报道，CAPS在APS患者中的发生率为0.8%。关于CAPS心脏受累的病例报告非常少。常见的心脏表现包括瓣膜增厚和病变、冠状动脉疾病以及微血管血栓形成导致的心肌梗死。在此，我们报告一例与心力衰竭相关的CAPS病例，并对类似病例进行文献综述。
一名有APS病史的24岁女性出现呼吸急促和右侧胸膜炎性胸痛。计算机断层扫描肺动脉造影显示右肺有新的肺栓塞。5天后，她出现高热，感染相关检查结果为阴性，伴有肺水肿的急性低氧性呼吸衰竭、休克、急性肾损伤，经胸超声心动图显示射血分数降低和整体运动减弱。鉴于出现多器官功能衰竭、一周内出现症状、存在抗磷脂抗体且排除了其他病因，诊断为CAPS。患者经静脉注射类固醇、血浆置换治疗后有显著改善，出院时口服逐渐减量的泼尼松并居家进行抗凝治疗。
CAPS可伴有多种心脏并发症，包括充血性心力衰竭、急性冠状动脉综合征、瓣膜病变和血栓形成。CAPS的心力衰竭管理包括静脉注射免疫球蛋白、血浆置换和皮质类固醇的三联疗法，而非传统治疗方法。
