Influence of female sex and fertile age on neuromyelitis optica spectrum disorders.
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Borisow Nadja, Kleiter Ingo, Gahlen Anna, Fischer Katrin, Wernecke Klaus-Dieter, Pache Florence, Ruprecht Klemens, Havla Joachim, Krumbholz Markus, Kümpfel Tania, Aktas Orhan, Ringelstein Marius, Geis Christian, Kleinschnitz Christoph, Berthele Achim, Hemmer Bernhard, Angstwurm Klemens, Weissert Robert, Stellmann Jan-Patrick, Schuster Simon, Stangel Martin, Lauda Florian, Tumani Hayrettin, Mayer Christoph, Zeltner Lena, Ziemann Ulf, Linker Ralf A, Schwab Matthias, Marziniak Martin, Then Bergh Florian, Hofstadt-van Oy Ulrich, Neuhaus Oliver, Winkelmann Alexander, Marouf Wael, Rückriem Lioba, Faiss Jürgen, Wildemann Brigitte, Paul Friedemann, Jarius Sven, Trebst Corinna, Hellwig Kerstin, NEMOS (Neuromyelitis Optica Study Group)
Аннотация
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.