Coexistence of Autoimmune Encephalitis and Other Systemic Autoimmune Diseases

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.

Serum Systemic Autoantibodies in Anti-N-Methyl-D-Aspartate Receptor Encephalitis

“…There were no patients diagnosed comorbidities with systemic autoimmune diseases. Our results have some different from the recent paper conducted by Zhao et al, which found anti-NMDAR encephalitis can coexist with non-organspecific autoimmune diseases, such as lupus (15). The paper also found that autoimmune diseases were more frequent in anti-leucine-rich glioma-inactivated 1 encephalitis than in anti-NMDAR encephalitis, and that autoimmune comorbidities did not affect the clinical course of AE.…”

Case report: anti-N-Methyl-D-Aspartate receptor encephalitis and bilateral temporal calcifications

“…Similarly, CEC is an autoimmune disease that can affect different organ systems to varying extents [ 21 ]. The pathogeneses of these two conditions may be highly homologous, which is in agreement with previous reports in the literature of the co-existence of autoimmune diseases and anti-NMDAR encephalitis [ 22 , 23 ]. The condition of our patient was complicated.…”

Anti-N-methyl-D-aspartate receptor encephalitis in a young female with subclinical hypothyroidism associated with anti-thyroid peroxidase and anti-thyroglobulin antibodies: A case report

“…In addition, they also report concomitant ADs such as systemic lupus erythematosus, chronic urticaria, and anaphylactoid purpura. 10 In our literature review, we found two cases which were misdiagnosed for HE which consequently led to the delay in the final diagnosis of anti-NMDAR encephalitis by 6 months to 4 years in an 18-year-old female and 14-year-old girl, respectively. 11,12 We minimized delays in both assessment and treatment by repeating LP on day 7 to test the CSF for anti-NMDAR antibodies because it is more sensitive than serum antibodies (100 versus 85.6% sensitivity).…”

“…In addition, they also report concomitant ADs such as systemic lupus erythematosus, chronic urticaria, and anaphylactoid purpura. 10 …”