Arnautovic Jelena Z, Yamasaki Hiroshi, Rosman Howard S
Division of Cardiovascular Medicine, St. John Providence Ascension Health System, 11800 Twelve Mile Rd, Warren, MI, USA.
Eur Heart J Case Rep. 2018 Aug 17;2(3):yty094. doi: 10.1093/ehjcr/yty094. eCollection 2018 Sep.
Libman-Sacks endocarditis (LSE) is an infrequently recognized pathogenesis of embolic cerebrovascular disease. Patients often have asymptomatic valvular dysfunction which if not recognized promptly, can lead to serious complications such as heart failure, arrhythmias, cerebroembolic phenomena with increased neurocognitive disability, and even death. It can be associated with systemic lupus erythematosus and/or antiphospholipid antibody syndrome (APLS).
Previously very healthy and active, 49-year-old Caucasian female with past medical history of mild lupus, for which she stopped treatment 10 year ago, saw a primary care physician complaining of intermittent double vision of 2 months duration. Urgent brain magnetic resonance imaging revealed multiple embolic infarcts of the brain stem. Further comprehensive work-up led to diagnosis of mitral LSE and APLS. After 2 months of systemic anticoagulation with warfarin and immunosuppressive therapy with hydroxychloroquine sulfate, repeat imaging demonstrated resolution of the mitral valve vegetation with no clinical recurrence of thromboembolic events at 6 months.
Mild, often silent, autoimmune disease as described in our case can lead to significant cerebrovascular disease. Patients who present with cryptogenic strokes with high suspicion of underlying autoimmune disease should be worked up thoroughly for possible valvular heart disease associated with lupus, APLS, or both. Acquisition of transoesophageal images proved superior to transthoracic approach and it should be implemented in these subsets of patients. With this case report, we highlight the importance of early recognition of cardiac manifestations, amelioration of risk factors, as well as close follow-up of lupus or APLS patients, as crucial steps in reducing their morbidity and mortality along with preventing recurrence or progression of cerebrovascular disease.
Libman-Sacks心内膜炎（LSE）是一种较少被认识到的栓塞性脑血管病发病机制。患者常有无症状性瓣膜功能障碍，若未及时识别，可导致严重并发症，如心力衰竭、心律失常、伴有神经认知功能障碍加重的脑栓塞现象，甚至死亡。它可与系统性红斑狼疮和/或抗磷脂抗体综合征（APLS）相关。
一名49岁的白种女性，既往非常健康且活跃，有轻度狼疮病史，10年前停止治疗，因主诉持续2个月的间歇性复视就诊于初级保健医生。紧急脑部磁共振成像显示脑干多发栓塞性梗死。进一步全面检查后诊断为二尖瓣LSE和APLS。在使用华法林进行2个月的全身抗凝及硫酸羟氯喹进行免疫抑制治疗后，重复成像显示二尖瓣赘生物消失，6个月时血栓栓塞事件无临床复发。
如我们病例中所述的轻度、通常无症状的自身免疫性疾病可导致严重的脑血管疾病。对于高度怀疑潜在自身免疫性疾病的不明原因卒中患者，应全面检查是否存在与狼疮、APLS或两者相关的可能瓣膜性心脏病。获取经食管图像被证明优于经胸途径，应在这些患者亚组中实施。通过本病例报告，我们强调早期识别心脏表现、改善危险因素以及对狼疮或APLS患者进行密切随访的重要性，这是降低其发病率和死亡率以及预防脑血管疾病复发或进展的关键步骤。
