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Impact of comorbid Sjögren syndrome in anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorders

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Abstract

Background

Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune neurological diseases of the central nervous system, which are characterized by the presence of serum anti-aquaporin-4 autoantibodies (AQP4-IgG). An association between Sjögren syndrome (SjS) and AQP4-IgG-positive NMOSD has been proposed, but the rate of coexistence has not been determined.

Methods

In this study, 4,447 patients suspected of having NMOSD with acute neurological episodes were evaluated for the positivity of serum AQP4-IgG, serum SS-A/Ro antibody, and the presence of SjS-related symptoms (dry eye, dry mouth).

Results

Of the 4,447 patients, 1,651 were positive for serum AQP4-IgG, and the remaining 2,796 were negative. A significantly higher proportion of AQP4-IgG-positive patients were positive for serum anti-SSA/Ro antibody (26.3 vs. 4.5%; p < 0.0001) and anti-SSB/La antibody (7.2 vs. 1.2%; p < 0.0001) and had dry eye (9.1 vs .4.9%; p < 0.0001) and dry mouth symptoms (8.9 vs. 3.7%; p < 0.0001). More than 80% of the patients with SjS with acute neurological events such as myelitis or optic neuritis were AQP4-IgG positive. AQ4-IgG-positive patients with comorbid SjS showed a higher female rate (97.1 vs. 89.0%; p = 0.0062), a higher positivity rate for oligoclonal bands (15.4 vs. 7.5%; p = 0.029), and a higher relapse frequency (p = 0.027) than AQP4-IgG-positive patients without comorbid SjS.

Conclusions

The prevalence of SjS is higher among AQP4-IgG-positive than AQP4-IgG-negative patients, with the potential prevalence of 10–20% at the diagnosis of AQP4-IgG-positive NMOSD. Comorbid SjS is more prevalent in females, and it has a higher relapse frequency among AQP4-IgG-positive patients.

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Authors and Affiliations

  1. Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan

    Tetsuya Akaishi, Toshiyuki Takahashi, Tatsuro Misu, Yoshiki Takai, Shuhei Nishiyama & Masashi Aoki

  2. Department of Education and Support for Regional Medicine, Tohoku University Hospital, Sendai, Japan

    Tetsuya Akaishi, Michiaki Abe & Tadashi Ishii

  3. Department of Neurology, National Hospital Organization Yonezawa National Hospital, Yonezawa, Japan

    Toshiyuki Takahashi

  4. Department of Multiple Sclerosis Therapeutics, Fukushima Medical University, Fukushima, Japan

    Kazuo Fujihara

  5. Department of Neurology, Tohoku Medical and Pharmaceutical University, Sendai, Japan

    Juichi Fujimori & Ichiro Nakashima

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  1. Tetsuya Akaishi
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  2. Toshiyuki Takahashi
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  3. Kazuo Fujihara
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  4. Tatsuro Misu
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Correspondence to Tetsuya Akaishi.

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This study was approved by the Institutional Review Board of the Tohoku University School of Medicine. Written informed consent was obtained from all enrolled patients and was performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Akaishi, T., Takahashi, T., Fujihara, K. et al. Impact of comorbid Sjögren syndrome in anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorders. J Neurol 268, 1938–1944 (2021). https://doi.org/10.1007/s00415-020-10377-6

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  • DOI: https://doi.org/10.1007/s00415-020-10377-6

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