Distinct clinical patterns in AQP4-IgG- positive NMOSD patients vs. Seronegative: Insights from a single-center study

Andrés M Villa1, Analisa Manin1, Carla Seimandi1

  • 1División Neurología. Hospital Gral. de Agudos Dr. José María Ramos Mejía, Bs. As, Argentina; Centro Argentino de Neuroinmunología (CADENI). Facultad de Medicina, Universidad de Buenos Aires. Argentina.

Abstract

Insights

Aquaporin-4 immunoglobulin G (AQP4-IgG) positive Neuromyelitis Optica Spectrum Disorder (NMOSD) patients show distinct clinical patterns. AQP4-IgG-positive NMOSD patients exhibit greater female predominance, early adulthood onset, and a higher prevalence of other autoimmune diseases.

Area of Science:

  • Neurology
  • Immunology
  • Autoimmune Diseases

Background:

  • Neuromyelitis Optica Spectrum Disorder (NMOSD) exhibits distinct clinical courses based on Aquaporin-4 immunoglobulin G (AQP4-IgG) serostatus.
  • Understanding these differences is crucial for accurate diagnosis and management of NMOSD.

Purpose of the Study:

  • To evaluate and compare clinical characteristics between AQP4-IgG-seropositive and AQP4-IgG-seronegative NMOSD patients.
  • To identify specific demographic, clinical, and immunological differences in a single center in Argentina.

Main Methods:

  • A retrospective cross-sectional study was conducted involving 108 NMOSD patients in Buenos Aires, Argentina.
  • Data from 94 patients were analyzed, focusing on AQP4-IgG status, demographics, clinical course, and co-existing autoimmune diseases.

Main Results:

  • Of 94 analyzed patients, 77 (82%) were AQP4-IgG positive.
  • AQP4-IgG-positive patients showed a significantly higher female-to-male ratio (1:10 vs. 1:1.2), higher relapse rates (99% vs. 17.6%), and a greater prevalence of other autoimmune diseases (44% vs. 12%).
  • Early adulthood onset (EAO-NMOSD) was more common in the seropositive group (81% vs. 35%).

Conclusions:

  • AQP4-IgG-positive NMOSD patients present with a more pronounced autoimmune profile, characterized by female predominance, early adulthood onset, recurrent disease courses, and a high comorbidity with other autoimmune conditions.
  • These findings highlight the importance of AQP4-IgG testing for stratifying NMOSD patients and tailoring treatment strategies.