Lupus and NMOSD: The Blending of Humoral Autoimmunity.
Lupus and NMOSD: The Blending of Humoral Autoimmunity.
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DOI：
10.1155/2020/8820071
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发表时间：
2020
影响因子：
--
通讯作者：
Melamed E
中科院分区：
文献类型：
--
作者：
Ochi MGS;Shapiro SC;Melamed E
系统性红斑狼疮(SLE)是一种慢性自身免疫性疾病，可攻击身体任何器官。它可能与其他自身免疫性神经系统疾病并存，如视神经脊髓炎谱系障碍(NMOSD)。NMOSD，以前被称为Devic病，是一种中枢神经系统(CNS)的自身免疫性炎症性疾病，目标是脊髓、视神经和某些大脑区域。目前的大多数证据表明，NMOSD最好的描述是一种中枢神经系统星形细胞病变。虽然这些疾病共享几种免疫抑制治疗方案，但NMOSD的及时诊断至关重要，因为患者可能受益于针对NMOSD而不是SLE的专门治疗。类固醇、血浆置换、静脉免疫球蛋白、环磷酰胺、硫唑嘌呤、霉酚酸酯和利妥昔单抗均用于治疗SLE和NMOSD。然而，最近有几种新疗法(inebilizumab、eculizumab和satralizumab)被专门批准用于NMOSD。在这一系列的病例中，我们报告了三名同时患有SLE和NMOSD的患者。我们描述了一名31岁的女性，在接受流感疫苗接种的情况下，11年的临床缓解后，她遭受了NMOSD发作；她的系统性红斑狼疮在羟基氯喹治疗下保持静止。接下来，我们描述了一位52岁的女性，在接受静脉注射环磷酰胺、口服类固醇和羟氯喹积极治疗SLE的过程中，出现了神经学破坏性血清阳性的NMOSD。最后，我们描述了一位48岁的女性，在用硫唑嘌呤和羟氯喹很好地控制了SLE的情况下，出现了血清阴性的NMOSD。这些病例说明了当NMOSD与SLE共存时，准确诊断和靶向治疗的重要性。
Systemic lupus erythematous (SLE) is a chronic autoimmune disease that can target any organ of the body. It may coexist with other autoimmune neurologic conditions such as neuromyelitis optica spectrum disorder (NMOSD). NMOSD, previously known as Devic's disease, is an autoimmune inflammatory disorder of the central nervous system (CNS) that targets the spinal cord, optic nerves, and certain brain regions. Most current evidence suggests that NMOSD is best described as a CNS astrocytopathy. While these diseases share several immunosuppressive treatment options, timely diagnosis of NMOSD is critical as patients may benefit from treatment tailored specifically to NMOSD as opposed to SLE. Steroids, plasmapheresis, intravenous immunoglobulin, cyclophosphamide, azathioprine, mycophenolate mofetil, and rituximab are used to treat both SLE and NMOSD. However, there are several new therapies (inebilizumab, eculizumab, and satralizumab) recently approved specifically for use in NMOSD. In this case series, we report on three patients with coexisting SLE and NMOSD. We describe a 31-year-old woman who suffered an NMOSD flare after 11 years of clinical remission in the context of receiving an influenza vaccination; her SLE remained quiescent on hydroxychloroquine. Next, we describe a 52-year-old woman with emergence of neurologically devastating seropositive NMOSD in the setting of active treatment for SLE with intravenous cyclophosphamide, oral steroids, and hydroxychloroquine. Last, we describe a 48-year-old woman with emergence of seronegative NMOSD in the setting of SLE that was well-controlled on azathioprine and hydroxychloroquine. These cases illustrate the importance of accurate diagnosis and targeted treatment of NMOSD when coexisting with SLE.