Neuromyelitis Optica and Non–Organ-Specific Autoimmunity

Abstract

Background: Neuromyelitis optica (NMO) is often associated with other clinical or serological markers of non-organ-specific autoimmunity. Objective: ToevaluatetherelationshipbetweenNMOspectrum disorders (NMOSDs), including NMO, longitudinally extensive transverse myelitis, and recurrent optic neuritis, and autoimmune disease. We concentrated on the association with systemic lupus erythematosus (SLE), Sjögren syndrome (SS), or serological evidence of these disorders, which commonly is a source of diagnostic confusion.

Myelitis in systemic lupus erythematosus: clinical features, immunological profile and magnetic resonance imaging of five cases

“…12,19,20 Whereas the AQP4-ab detection is a specific marker that distinguishes neuromyelitis optica (NMO) from other etiologies, in these patients, coexisting pathologies is postulated. 19 In two of our patients have been tested for the presence of these antibodies by IFI with negative results. The sensitivity depends on the method of detection: assay based on cells transfected with AQP4-M23 isoform were the most sensitive but is not widely available.…”

Efficacy of tacrolimus in Sjögren’s syndrome-associated CNS disease with aquaporin-4 autoantibodies

“…Approximately 3% of patients with NMO have coexisting SLE or SjS, and CNS-SjS patients with optic nerve neuritis or longitudinal myelitis (conditions called "NMO spectrum disorder") often present positive for AQP4-Ab [4,6,7]. To our knowledge, this is the first reported assessment of tacrolimus in patients with CNS-SjS with AQP4-Ab.…”

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

“…Inflammatory myelitis can be further divided depending on whether gray or white matter is primarily involved [30]. High disease activity is associated with gray matter dysfunction and overt inflammation, whereas antiphospholipid and NMO-IgG/ antiaquaporin 4 antibodies, along with lower disease activity, correlate with white matter dysfunction [30,31]. Optic neuritis can occur without myelitis and it is either inflammatory or ischemic (associated with antiphospholipid antibody positivity) [5].…”

“…Transverse myelitis has also been described in patients with pSS [3,82,83], and in a small percentage it may be accompanied by brain MS-like lesions or by optic neuritis making, at times, the distinction from a primary demyelinating disease such as MS or NMO rather difficult [31,80]. The meninges can also be affected, alone or in the context of meningoencephalitis [84], while in some cases a subacute encephalopathy presenting with memory loss, cognitive dysfunction, visual disturbances, and reduced concentration and attention has been noted [80].…”