PDF(Pubmed)
Abstract：
The coexistence of two or more autoimmune diseases is well-known, e.g., a person can have neuromyelitis optica (NMO) and systemic lupus erythematosus (SLE) at the same time. We report a case of NMO-SLE overlap syndrome with myelitis and myocarditis as the initial manifestations. The patient, a 64-year-old man, presented with a 15-day history of ascending sensory loss and a 10-day history of exertional dyspnea. Magnetic resonance imaging (MRI) revealed longitudinally extensive transverse myelitis (LETM) from C7 to T6. Serology showed a high anti-aquaporin-4 antibody level. We diagnosed NMO based on these findings. Echocardiography showed a hypokinetic left ventricle with a severely reduced ejection fraction. Cardiac MRI demonstrated delayed gadolinium enhancement in the myocardium consistent with active inflammation. Because the cardiac findings could not be explained on the basis of NMO, we started searching for another autoimmune disease. Serology came back positive for a variety of autoantibodies, including antinuclear, anti-dsDNA, anti-chromatin, anti-cardiolipin, anti-β2-glycoprotein-1, and lupus anticoagulant. These findings, along with leukopenia and low serum complement C4, prompted us to diagnose SLE, in addition to NMO. He was initially treated with plasmapheresis and methylprednisolone. Maintenance therapy consisted of rituximab, hydroxychloroquine, and aspirin. One year later, he only complained of mild paresthesia in the feet. Patients with NMO should always be screened for SLE especially if they have signs and symptoms that cannot be accounted for by NMO alone, e.g., our patient had myocarditis. Conversely, patients with SLE and evidence of transverse myelitis should be screened for anti-AQP4 antibodies.
摘要：
两种或多种自身免疫性疾病的共存是众所周知的，例如，一个人可以同时患有视神经脊髓炎（NMO）和系统性红斑狼疮（SLE）。我们报告一例NMO-SLE重叠综合征，以脊髓炎和心肌炎为首发表现。病人,一个64岁的男人,有15天的感觉减退史和10天的劳力性呼吸困难史。磁共振成像（MRI）显示从C7到T6的纵向广泛的横贯性脊髓炎（LETM）。血清学显示高抗水通道蛋白4抗体水平。我们根据这些发现诊断了NMO。超声心动图显示左心室运动不足，射血分数严重降低。心脏MRI显示心肌中a的延迟增强与活动性炎症一致。因为无法根据NMO解释心脏检查结果，我们开始寻找另一种自身免疫性疾病。血清学对多种自身抗体呈阳性，包括反核,抗dsDNA，抗染色质,抗心磷脂,抗β2-糖蛋白-1和狼疮抗凝药。这些发现,伴随着白细胞减少和低血清补体C4，促使我们诊断SLE，除了NMO。他最初接受血浆置换和甲基强的松龙治疗。维持治疗包括利妥昔单抗，羟氯喹,还有阿司匹林.一年后,他只抱怨脚上有轻微的感觉异常。应始终对NMO患者进行SLE筛查，尤其是如果他们的体征和症状不能单独由NMO解释。例如，我们的病人有心肌炎.相反，有横贯性脊髓炎证据的SLE患者应进行抗AQP4抗体筛查.
