Abstract
Systemic lupus erythematosus (SLE) is an autoimmune disease that can have detrimental effects on many different systems in the body, including the central nervous system. Neuropsychiatric SLE (NPSLE) refers to several different neurological and/or behavioral clinical syndromes, and has been reported as having a prevalence rate of approximately 30−40%, while manifestation of myelitis or optic neuritis of NPSLE is rare (~1%). Myelitis and optic neuritis are easily identifiable since myelitis is frequently transverse, and manifests as severe disturbances in both motor and sensory pathways, while optic neuritis is often both bilateral and severe. At least 85% of patients experience relapses in the form of optic neuritis, transverse myelitis, or both. Furthermore, some cases of NPSLE with optic neuritis are often complicated by myelitis. Interestingly, the characteristics of myelitis or optic neuritis in NPSLE are quite similar to neuromyelitis optica (NMO), a disease characterized by bilateral optic neuropathy and transverse myelopathy. In fact, magnetic resonance imaging (MRI) of patients with NPSLE has demonstrated longitudinal spinal involvement showing cord swelling and hyperintense lesions in central regions. These findings are also typically observed in MRIs of patients with NMO. Additionally, anti-aquaporin 4 (AQP4) antibodies have been discovered in patients with NMO and with NPSLE with myelitis and/or optic neuritis. Therefore, complications that are often encountered with NMO should be considered when treating cases of NPSLE with myelitis and/or optic neuritis. Moreover, since the treatment of NMO closely resembles the therapeutic approaches taken for NPSLE, corticosteroids alone or in combination with immunosuppressants could prove effective in reducing incidents of relapse. Some patients, however, may be refractory to steroid therapy; in such cases, plasma exchange may have priority over other second-line therapeutic strategies, such as intravenous immunoglobulin and rituximab, because of treatment approaches typically employed in NMO. In this review, I will discuss pathological similarities between NPSLE with myelitis and/or optic neuritis and NMO with the aim of demonstrating that our knowledge of NMO should be considered when treating NPSLE with myelitis and/or optic neuritis.
Highlights
Systemic lupus erythematosus (SLE) is an autoimmune disease that can have detrimental effects on the central nervous system (CNS)
neuromyelitis optica (NMO) first described by Devic in 1894 is a disease characterized by bilateral optic neuropathy and transverse myelopathy, which can be pathologically identified by severe demyelination and necrotic changes [14]
T2weighted magnetic resonance imaging (MRI) of spinal cords in patients with Neuropsychiatric SLE (NPSLE) and myelitis often exhibit cord swelling and hyperintense signals that are localized in the central part of the spinal cord
Summary
Systemic lupus erythematosus (SLE) is an autoimmune disease that can have detrimental effects on the central nervous system (CNS). In a 3-year prospective study of 370 patients with SLE and no history of CNS complication, 16/370 patients were reported to develop major CNS-related events including myelopathy (4/16), optic neuritis (1/16), and positive neuromyelitis optica (NMO)-IgG antibodies (2/16) [3]. Anti-aquaporin-4 (AQP4) antibodies were discovered in patients with NMO [10] and in patients with NPSLE, especially with myelitis or optic neuritis [11,12,13]. The relevance of this antibody will be discussed
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