Influence of female sex and fertile age on neuromyelitis optica spectrum disorders

Abstract

Background: Gender and age at onset are important epidemiological factors influencing prevalence, clinical presentation, and treatment response in autoimmune diseases. Objective: To evaluate the impact of female sex and fertile age on aquaporin-4-antibody (AQP4-ab) status, attack localization, and response to attack treatment in patients with neuromyelitis optica (NMO) and its spectrum disorders (neuromyelitis optica spectrum disorder (NMOSD)). Methods: Female-to-male ratios, diagnosis at last visit (NMO vs NMOSD), attack localization, attack treatment, and outcome were compared according to sex and age at disease or attack onset. Results: A total of 186 NMO/SD patients (82% female) were included. In AQP4-ab-positive patients, female predominance was most pronounced during fertile age (female-to-male ratio 23:1). Female patients were more likely to be positive for AQP4-abs (92% vs 55%; p < 0.001). Interval between onset and diagnosis of NMO/SD was longer in women than in men (mean 54 vs 27 months; p = 0.023). In women, attacks occurring ⩽40 years of age were more likely to show complete remission (p = 0.003) and better response to high-dose intravenous steroids (p = 0.005) compared to woman at >40 years. Conclusion: Our data suggest an influence of sex and age on susceptibility to AQP4-ab-positive NMO/ SD. Genetic and hormonal factors might contribute to pathophysiology of NMO/SD.

Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis

“…Disease onset ranges between 4 and 88 years with a mean age at onset of 39 years ( 18 – 21 ). Women are disproportionately more often affected and, particularly in AQP4-seropositive patients, female to male-ratio can reach up to 10:1 ( 19 , 22 , 23 ). In 20–30% of patients, depending on the assay used, AQP4-Abs are not detectable ( 24 , 25 ).…”

Vaccines and the association with relapses in patients with neuromyelitis optica spectrum disorder

“…Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing autoimmune disease that preferentially targets the spinal cord and optic nerves, leading to paralysis and blindness. Risk factors that predict a higher risk of relapse include female sex, African ancestry, positive aquaporin-4 antibody (AQP4-IgG) serostatus, and lack of preventive immunosuppression (Mealy et al, 2012, Kimbrough et al, 2014, Borisow et al, 2016, Wingerchuk et al, 2007. Activation of the immune system from infections increases risk of relapse in multiple sclerosis (MS), (McKay et al, 2016) may increase the risk in NMOSD (Jarius et al, 2012), and increases morbidity and mortality in other rheumatologic diseases (Sfriso et al, 2010, Doria et al, 2008, Wolfe et al, 1994.…”

Neuroimmunological Registries in Germany

“…This difficult diagnosis following a somewhat circuitous route was documented in an article on patients who initially underwent a spinal biopsy (which was in some cases not acceptably tolerated) for a suspect-ed malignant spinal tumor, but who in the end had apparent tumefactive longitudinal extensive transverse myelitis (LETM) [17]. Furthermore, patients with unusual manifestations of NMOSD in late life stages were described using the NEMOS database [18]; additionally the influence of gender on clinical aspects of NMSOD was characterized [19]. In 2015 NEMOS was able to make a substantial contribution regarding acute therapy of NMSOD relapse by demonstrating the early importance of plasma separation/immune adsorption in particular for longitudinal extensive transverse myelitis (LETM) [20].…”