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Original Research ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Neurol. | doi: 10.3389/fneur.2019.01142

Coexistence of autoimmune encephalitis and other systemic autoimmune diseases

 Jing Zhao1,  Hongzhi Guan2*,  Jiewen Zhang3*, Cancan Wang4, Xiaolu Xu2, Yuanxing Zhang1, Haitao Ren2, Zhixia Ren1 and Gai Li1
  • 1People's Hospital of Zhengzhou University, China
  • 2Peking Union Medical College Hospital (CAMS), China
  • 3People's Hospital of Zhengzhou University, China
  • 4People's Hospital of Beijing Daxing District, Capital Medical University, China

Abstract
Background: In recent years, the phenomenon of coexisting systemic autoimmune diseases (ADs) in patients with autoimmune encephalitis (AE) has been increasingly found, while its clinical significance remains unexplored. This study aimed to investigate the types and potential clinical associations of autoimmune comorbidities in patients with antibody-positive AE.
Methods: A retrospective cohort study of patients with antibody-positive AE was conducted from 2011 to 2018. The demographics, clinical characteristics, and follow-up data were reviewed.
Results: We enrolled 517 patients, among whom 45 were affected by one or more types of ADs, including Hashimoto's thyroiditis (HT) (n=28), systemic lupus erythematosus (SLE) (n=3), anaphylactoid purpura (n=3), vitiligo (n=3), Sjögren’s syndrome (SS) (n=2), chronic urticaria (n=2), bullous pemphigoid (n=1), uveitis (n=1), myasthenia gravis (MG) (n=1), and the coexistence of SLE and anaphylactoid purpura (n=1). The proportion of patients with coexisting ADs was higher in those with anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis than in those with anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis (13/111 vs 16/307) (P=0.021). In anti-NMDAR and anti-LGI1 encephalitis patients, there were no significant differences in the age at onset, sex ratio, proportion of patients with tumors, disease severity, or recurrence between the groups with and without ADs.
Conclusions: One or more types of ADs developed in AE patients, and patients with anti-LGI1 encephalitis had a higher frequency of autoimmune comorbidities than those with anti-NMDAR encephalitis. And we found that autoimmune comorbidities did not affect the clinical course of AE.

Keywords: Autoantibodies, Encephalitis, autoimmune encephalitis, Autoimmune Diseases, comorbidities

Received: 22 Jun 2019; Accepted: 11 Oct 2019.

Copyright: © 2019 Zhao, Guan, Zhang, Wang, Xu, Zhang, Ren, Ren and Li. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence:
Prof. Hongzhi Guan, Peking Union Medical College Hospital (CAMS), Beijing, 100730, Beijing Municipality, China, guanhz@263.net
Prof. Jiewen Zhang, People's Hospital of Zhengzhou University, zhengzhou, China, zhangjiewen9900@126.com