Lupus and NMOSD: The Blending of Humoral Autoimmunity

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.

Clinical features of neuromyelitis optica combined with or without connective tissue disease

“…And in addition to AQP4-IgG, 70% of NMOSD patients produce at least one other auto-antibodies, such as anti-SSA antibodies, anti-SSB antibodies, which implies a high rate of co-integrated autoimmune diseases in NMOSD. 9 In this work, 11/16 (68.75%) of 16 patients were positive for AQP4-IgG, except for four cases in which AQP4-IgG testing was not performed due to objective factors, by the contents of previously reported kinds of literature. MRI of SLE patients positive for AQP4-IgG may have distinct imaging characteristics with extensive cerebral lesions of white matter, which was partially speci c for MRI ndings in NMOSD patients.…”

Combination of Neuromyelitis Optica Spectrum Disorder with Myasthenia Gravis, Systemic Lupus Erythematosus and Hashimoto's Thyroiditis: A Case of Multiple Autoimmune Syndrome

“…5 Sjogren's syndrome and SLE are the most frequently reported comorbid systemic autoimmune diseases and MG is the most reported neurological disease accompanying NMOSD. [5][6][7] The pathological mechanism of the SLE and MG association is not clearly known. SLE may develop after thymectomy in MG patients, but there was no thymectomy history in our case since there was no thymoma.…”

Clinical Reasoning: A 40-Year-Old Woman Presenting With Encephalopathy and Paraparesis

“…[9] First-line treatment for SLE and NMOSD is identical, which involves high-dose steroids and plasma exchange. [5] The use of intravenous immunoglobulin (IVIG) in addition to highdose intravenous steroids has also been shown to be superior to high-dose intravenous steroids alone [10,11], but IVIG alone is not recommended. [11] However, while acute treatment for both diseases overlaps, long-term treatment and immunosuppression can differ.…”

“…For instance, hydroxychloroquine is used in patients with SLE to reduce disease flares, but not in NMOSD. [5] Additionally, using steroid-sparing agents may be influenced by coexisting end-organ damage seen with SLE associated with NMOSD. For example, in patients with lupus nephritis without NMOSD, the drug of choice would be Cyclophosphamide or Mycophenolate Mofetil (MMF), but in patients with lupus nephritis with NMOSD, Cyclophosphamide has been shown to be ineffective.…”

“…[4] Rituximab was started as maintenance therapy, anticipating she would test positive for NMOSD, as the two autoimmune diseases can coexist. [5] Her serum anti-Myelin Oligodendrocyte Glycoprotein (MOG) and anti-Aquaporin 4 (AQP4) antibodies sent before we started PLEX returned negative.…”