Nadja Borisow1, Ingo Kleiter2, Anna Gahlen2, Katrin Fischer3, Klaus-Dieter Wernecke4, Florence Pache1, Klemens Ruprecht5, Joachim Havla6, Markus Krumbholz6, Tania Kümpfel6, Orhan Aktas7, Marius Ringelstein7, Christian Geis8, Christoph Kleinschnitz9, Achim Berthele10, Bernhard Hemmer11, Klemens Angstwurm12, Robert Weissert12, Jan-Patrick Stellmann13, Simon Schuster14, Martin Stangel15, Florian Lauda16, Hayrettin Tumani17, Christoph Mayer18, Lena Zeltner19, Ulf Ziemann19, Ralf A Linker20, Matthias Schwab21, Martin Marziniak22, Florian Then Bergh23, Ulrich Hofstadt-van Oy24, Oliver Neuhaus25, Alexander Winkelmann26, Wael Marouf27, Lioba Rückriem28, Jürgen Faiss3, Brigitte Wildemann29, Friedemann Paul30, Sven Jarius29, Corinna Trebst31, Kerstin Hellwig2. 1. NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center, Department of Neurology, Charité University Medicine Berlin, Berlin, Germany. 2. Department of Neurology, St. Josef Hospital, Ruhr University Bochum, Bochum, Germany. 3. Department of Neurology, Asklepios Fachklinikum Teupitz, Teupitz, Germany. 4. CRO SOSTANA GmbH and Charité University Medicine Berlin, Berlin, Germany. 5. Department of Neurology and Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine Berlin, Berlin, Germany. 6. Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University of Munich, Munich, Germany. 7. Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf, Germany. 8. Department of Neurology, University Hospital of Würzburg, Würzburg, Germany/Hans-Berger Department of Neurology and Center for Sepsis Control and Care (CSCC), Jena University Hospital, Jena, Germany. 9. Department of Neurology, University Hospital Essen, Essen, Germany. 10. Department of Neurology, Klinikum rechts der Isar, Technische Universität München, Munich, Germany. 11. Department of Neurology, Klinikum rechts der Isar, Technische Universität München, Munich, Germany/Munich Cluster for Systems Neurology (SyNergy), Technische Universität München, Munich, Germany. 12. Department of Neurology, University Hospital Regensburg, Regensburg, Germany. 13. Institute of Neuroimmunology and MS (INIMS), University Medical Center Hamburg-Eppendorf, Hamburg, Germany/Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 14. Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 15. Department of Clinical Neuroimmunology and Neurochemistry and Department of Neurology, Hannover Medical School, Hannover, Germany. 16. Department of Neurology, University of Ulm, Ulm, Germany. 17. Department of Neurology at RKU and Specialty Clinic of Neurology Dietenbronn, University of Ulm, Ulm, Germany. 18. Department of Neurology, Goethe University Frankfurt, Frankfurt, Germany. 19. Department of Neurology and Stroke and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany. 20. Department of Neurology, Friedrich-Alexander University Erlangen-Nüremberg, Erlangen, Germany. 21. Hans-Berger Department of Neurology, Jena University Hospital, Jena, Germany. 22. Department of Neurology, University of Münster, Münster, Germany/Department of Neurology and Neurological Intensive Care, Isar-Amper-Clinic, Munich-East, Haar, Germany. 23. Department of Neurology, Leipzig University, Leipzig, Germany. 24. Department of Neurology, Klinikum Bayreuth, Bayreuth, Germany/Department of Neurology, Klinikum Westfalen, Dortmund, Germany. 25. Department of Neurology, SRH Krankenhaus Sigmaringen, Sigmaringen, Germany. 26. Department of Neurology, University of Rostock, Rostock, Germany. 27. Department of Neurology, HELIOS Hanseklinikum Stralsund, Stralsund, Germany. 28. Department of Neurology, MediClin Hedon Klinik, Lingen, Germany. 29. Department of Neurology, Heidelberg University, Heidelberg, Germany. 30. NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center, Department of Neurology, Charité University Medicine Berlin, Berlin, Germany/Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine and Charité University Medicine Berlin, Berlin, Germany. 31. Department of Neurology, Hannover Medical School, Hannover, Germany.
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.
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.
Entities:
Keywords:
Neuromyelitis optica; age factors; aquaporin 4; sex
Authors: Sara Salama; Hazem Marouf; M Ihab Reda; Amal R Mansour; Osama ELKholy; Michael Levy Journal: J Neuroimmunol Date: 2018-08-29 Impact factor: 3.478
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