Abstract
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.
Highlights
Systemic lupus erythematous (SLE) is a chronic autoimmune disease with multiorgan involvement. e etiology of SLE is likely multifactorial with genetic, hormonal, immunologic, and environmental factors contributing. e reported prevalence of SLE is 20 to 150 cases per 100,000; in women, rates vary from 164 (Caucasian) to 406 (African American) per 100,000 [1,2,3]
neuromyelitis optica spectrum disorder (NMOSD), previously known as Devic’s disease, is a central nervous system (CNS) astrocytopathy with damage mediated by the humoral immune system [12,13,14,15]. e most common clinical features are optic neuritis, longitudinally extensive transverse myelitis (LETM), area postrema syndrome, and other brainstem and cerebral events
We describe three cases of coexistent SLE and NMOSD, with several differences in clinical presentation
Summary
SLE is a chronic autoimmune disease with multiorgan involvement. e etiology of SLE is likely multifactorial with genetic, hormonal, immunologic, and environmental factors contributing. e reported prevalence of SLE is 20 to 150 cases per 100,000; in women, rates vary from 164 (Caucasian) to 406 (African American) per 100,000 [1,2,3]. Case Reports in Rheumatology antibodies (MOG-IgG) has furthered increased our understanding in this regard [12, 17]. 20 to 30 percent of patients with clinical characteristics of NMOSD are AQP4-lgG negative, but 30 percent of those seronegative for AQP4-IgG have detectable MOG-IgG [16, 18]. E seronegative NMOSD subset of patients tend to be younger, less frequently female, more responsive to steroids, and less likely to relapse [16]. When SLE is active despite chronic hydroxychloroquine therapy, many other immunosuppressive agents may be added depending on organ involvement. As patients may have coexistent SLE and NMOSD, it is critical to consider both diseases when selecting therapy in order to ensure best clinical outcomes. In the following case series, we describe three patients with coexisting SLE and NMOSD and discuss the clinical management in each case
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