Polyautoimmunity and multiple autoimmune syndrome in patients with neuromyelitis optica spectrum disorder.

Mult Scler Relat Disord

Neurology Department, Centro Hospitalar Universitário de Santo António, Largo do Prof. Abel Salazar, Porto, 4099-001, Portugal; Unit for Multidisciplinary Research in Biomedicine (UMIB), Instituto de Ciências Biomédicas Abel Salazar - Universidade do Porto, Porto, Portugal; Laboratory for Integrative and Translational Research in Population Health, Porto, Portugal; Alexion, Astrazeneca Rare, Barcelona, Spain.

Published: October 2024

AI Article Synopsis

  • This study focused on comparing clinical and laboratory characteristics of neuromyelitis optica spectrum disorders (NMOSD) patients with autoimmune diseases (AID) versus those without AID.
  • Among the 46 NMOSD patients analyzed, 16 (34.8%) had polyautoimmunity, with most being women around 40 years old, and AID often preceded NMOSD symptoms by an average of 7 years.
  • The findings indicated that NMOSD patients with polyautoimmunity had a higher recurrence of their disease and more inflammatory cerebrospinal fluid, although both groups had similar initial clinical symptoms and no significant differences in long-term functional outcomes.

Article Abstract

Background: The coexistence of neuromyelitis optica spectrum disorders (NMOSD) with other autoimmune diseases (AID) has been increasingly reported. The prevalence and significance of this association are not fully understood.

Objectives: This study aimed to compare the clinical and laboratory characteristics in NMOSD patients with and without AID.

Methods: Retrospective cross-sectional observational study was conducted involving adults meeting NMOSD criteria followed in a neuroimmunology clinic at a tertiary center. Descriptive analysis of clinical/paraclinical/treatment/outcome data collected from the medical records was compared between NMOSD patients with AID (polyautoimmunity) and those without AID.

Results: From a cohort of 46 NMOSD patients, 16 (34.8 %) patients, mostly women around 40 years of age, presented with polyautoimmunity: 10 anti-AQP4 positive, 4 anti-MOG positive, and 2 seronegative. Five different organ -specific AID, and six systemic AID were identified in the polyautoimmunity patients group, in addition to 6 cases of multiple autoimmune syndrome. The AID manifestation preceded NMOSD in 10 (62.5 %) patients, with a median interval of 7 years. The NMOSD with polyautoimmunity and NMOSD without AID groups had similar initial clinical manifestations with optic neuritis and/or myelitis being most frequent. Inflammatory CSF, namely elevated proteins, was more common in the polyautoimmunity group (13.0 % in NMOSD vs. 31.3 % in NMOSD+AID, p = 0.003). After a 10±6 years follow-up period, more patients with polyautoimmunity had a relapsing disease (75.0 % in NMOSD vs. 46.7 % in NMOSD+AID, p = 0.012) but no difference in the functional outcome evaluated by the EDSS score was identified.

Conclusions: Polyautoimmunity was common in AQP4 positive NMOSD patients leading to a significantly higher risk of disease recorrence. The presence of polyautoimmunity and multiple autoimmune syndrome in NMOSD patients suggests the existence of common susceptibility factors or pathophysiological mechanisms, emphasizing the importance of a multidisciplinary approach to those patients.

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Source
http://dx.doi.org/10.1016/j.msard.2024.105848DOI Listing

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