原发性晚发型抗磷脂综合征1例

S. Salvucci, L. Manfredi, L. Gamba, G. Montozzi, R. Santangeli, G. Moroncini
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引用次数: 1

摘要

目的:抗磷脂综合征(APS)常见于年轻患者,很少见于老年患者。在这里,我们提出一个迟发性三阳性原发性APS以复发性血栓事件为特征的病例。方法2019年11月,一名80岁男性首次出现左腿深静脉血栓形成。接受氟达哌啶治疗1年,停药1个月后血栓复发。对血栓形成原因的研究显示抗磷脂抗体(LAC、抗心磷脂和高滴度抗β 2糖蛋白I IgM)呈阳性。此外,患者接受了一级诊断和内镜检查,排除了其他免疫原因、感染和恶性肿瘤。诊断为原发性APS,并开始华法林治疗,INR范围在2至3之间。2021年11月,患者在抗凝治疗后髂深静脉和股静脉血栓复发,引起了我们的注意。实验室检查证实抗磷脂抗体呈三倍高滴度阳性,全身CT扫描证实没有可疑的恶性病变。血清学研究与其他自身免疫性疾病的综合自身免疫小组均为阴性,超声心动图研究排除了心脏受累的存在。结果:虽然年龄已被证明是长期抗凝治疗患者严重出血发作的危险因素,但考虑到患者临床状况良好且无合并症,我们决定根据EULAR推荐开始羟氯喹和华法林(INR范围在2.5至3.5之间)和低剂量阿司匹林联合治疗。患者被告知疾病的性质和需要进行出血高风险的治疗。2021年12月,尽管接种了两剂疫苗,但他感染了COVID-19,并以预防剂量用低分子肝素替代华法林治疗,但几天后他出现呼吸困难和去饱和。肺部CT扫描显示双侧肺栓塞,可归因于并发病毒感染和APS(停用华法林后未完全控制),因此临床医生决定用华法林替代治疗。一个月后,患者出院,临床健康状况良好。结论由于老年人对迟发性APS缺乏认识,准确诊断和治疗迟发性APS是一项挑战。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
PO.3.69 A case of late onset of primary antiphospholipid syndrome
PurposeAntiphospholipid syndrome (APS) usually affects young patients and is rarely described in the elderly. Here, we present a case of late-onset of triple-positive primary APS characterized by recurrent thrombotic events.MethodsIn November 2019, an 80-year-old man presented the first deep vein thrombosis in his left leg. He received fondaparinux treatment for one year, but the thrombosis relapsed one month after stopping treatment. The search for thrombophilic causes has shown positivity for antiphospholipid antibodies (LAC, anticardiolipin, and anti-beta2 glycoprotein I IgM at high titres). In addition, the patient underwent first-level diagnostics and endoscopic examinations excluding other immunological causes, infections and malignancy. A diagnosis of primary APS was made and the warfarin therapy started with a range of INR between 2 and 3. In November 2021, the patient was brought to our attention following the recurrence of thrombosis of the deep iliac and femoral veins despite anticoagulant treatment. Laboratory tests confirmed the presence of triple high titre positivity of antiphospholipid antibodies and a total body CT scan confirmed the absence of suspected malignant lesions. Serological studies with a comprehensive autoimmunity panel for other autoimmune disorders were negative and an echocardiographic study ruled out the presence of cardiac involvement.ResultsAlthough age has been demonstrated to be a risk factor for severe bleeding episodes in patients placed on long-term anticoagulation, we decided to start hydroxychloroquine and combination therapy with warfarin (INR range between 2.5 and 3.5) and low-dose aspirin according to EULAR recommendations, considering the patient’s good clinical condition and absence of comorbidities. The patient was informed about the nature of the disease and the need for treatment with a high risk of bleeding. In December 2021, despite vaccination with two doses, he contracted COVID-19 infection and warfarin treatment was replaced with low molecular weight heparin at prophylactic dosage, but after some days he presented dyspnoea with desaturation. A lung CT scan showed bilateral pulmonary embolism, attributable to intercurrent viral infection and to APS (not completely controlled after warfarin withdrawal), and so clinicians decided to replace treatment with warfarin. After one month, patient had been discharged from hospital in good clinical health.ConclusionsAccurate diagnosis and treatment of late-onset APS represent a challenge due to the lack of knowledge of this disease in the elderly.
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