Abstract

BackgroundNeuromyelitis optica spectrum disorder (NMOSD) can coexist with non-organ-specific or organ-specific autoimmune diseases. The aim of this study was to investigate and compare the features between NMOSD without and with autoimmune diseases, and NMOSD with non-organ-specific and organ-specific autoimmune diseases.MethodsOne hundred and fifty five NMOSD patients without autoimmune diseases (n = 115) and with autoimmune diseases (n = 40) were enrolled. NMOSD with autoimmune diseases were divided by organ-specific autoimmune diseases. The clinical, laboratory and magnetic resonance imaging features between two groups were assessed.ResultsMotor deficit was less frequent in NMOSD patients with non-organ-specific autoimmune diseases (p = 0.024). Cerebrospinal fluid white blood cell and protein, serum C-reactive protein and immunoglobulin G were lower in NMOSD patients without autoimmune diseases, while several autoantibodies seropositivity and thyroid indexes were significantly higher in NMOSD patients with autoimmune diseases (p < 0.05). No difference was found in other clinical and laboratory characteristics between different NMOSD subtypes (p > 0.05). NMOSD patients with autoimmune diseases had higher brain abnormalities than NMOSD without autoimmune diseases (p < 0.001).ConclusionsThe characteristics between NMOSD without and with autoimmune diseases were similar. NMOSD with autoimmune diseases have high frequency of brain abnormalities.

Highlights

  • Neuromyelitis optica spectrum disorder (NMOSD) can coexist with non-organ-specific or organ-specific autoimmune diseases

  • A total of 155 patients satisfied the diagnostic criteria for inclusion in this study: 115 NMOSD patients without autoimmune diseases and 40 with autoimmune diseases (22 NMOSD patients with non-organ-specific autoimmune diseases and 18 NMOSD patients with organ-specific autoimmune diseases)

  • Motor deficit was less frequent in NMOSD patients with non-organ-specific autoimmune diseases than in NMOSD patients with organspecific autoimmune diseases (p = 0.024)

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Summary

Introduction

Neuromyelitis optica spectrum disorder (NMOSD) can coexist with non-organ-specific or organ-specific autoimmune diseases. The aim of this study was to investigate and compare the features between NMOSD without and with autoimmune diseases, and NMOSD with non-organ-specific and organ-specific autoimmune diseases. Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system that affects the optic nerve and spinal cord but has protean and diverse potential clinical and radiological manifestations [1,2,3]. The broadened array of disorders associated with NMO immunoglobulin G (IgG) has been termed ‘NMO spectrum disorders’ (NMOSD), the diagnosis of which is greatly facilitated by the association of NMOSD with a specific biomarker for NMO, NMO-IgG [1]. Several groups have recognized a strong association of NMOSD with nonorgan-specific autoimmune diseases (e.g. systemic lupus erythematosus (SLE), Sjögren syndrome (SS), rheumatoid

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