PMID- 38546185 OWN - NLM STAT- MEDLINE DCOM- 20240329 LR - 20240329 IS - 1526-632X (Electronic) IS - 0028-3878 (Linking) VI - 102 IP - 5 DP - 2024 Mar 12 TI - Mortality of the Danish Nationwide AQP4 Antibody-Seropositive Neuromyelitis Optica Spectrum Disorder Patient Cohort. PG - e209147 LID - 10.1212/WNL.0000000000209147 [doi] AB - BACKGROUND AND OBJECTIVES: We aimed to evaluate the mortality of patients with AQP4 antibody-seropositive (AQP4-Ab+) neuromyelitis optica spectrum disorder (NMOSD) in Denmark compared with that in the general population. METHODS: We identified patients with AQP4-Ab+ NMOSD fulfilling the 2015 International Panel for Neuromyelitis Optica Diagnosis (IPND) criteria from multiple sources (laboratories and the Danish Multiple Sclerosis Registry). We obtained detailed information about patients from hospital records and about the general population matched on age, sex, and calendar year from Statistics Denmark. We calculated standardized mortality ratio (SMR), excess number of deaths per 1,000 person-years (EDR), and life expectancies compared with those of the matched general population. We examined predictive factors of mortality and the cause of death. RESULTS: Of 66 patients with AQP4-Ab+ NMOSD between 2008 and 2020, 15 died. Overall, the SMR was 2.54 (95% CI 1.47-4.09), and the EDR was 16.8 (95% CI 4.6-34.3). The median life expectancy for patients with AQP4-Ab+ NMOSD was 64.08 years (95% CI 53.02-83.9), compared with 83.07 years for the general population. Risk of death over time was increased in the patient population with a hazard ratio (HR) of 2.22 (1.34-3.68; p = 0.002). The cause of death was directly related to NMOSD in 93% of the cases. The age at disease onset was an independent predictor of death (HR 1.042; 95% CI 1.006-1.079; p = 0.02). DISCUSSION: AQP4-Ab+ NMOSD is associated with increased mortality and shorter life expectancy compared with that in the general population, underlining the need for highly effective treatment approaches. FAU - Papp, Viktoria AU - Papp V AD - From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark. FAU - Magyari, Melinda AU - Magyari M AUID- ORCID: 0000-0002-0972-5222 AD - From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark. FAU - Moller, Soren AU - Moller S AUID- ORCID: 0000-0003-0858-4269 AD - From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark. FAU - Sellebjerg, Finn AU - Sellebjerg F AUID- ORCID: 0000-0002-1333-9623 AD - From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark. FAU - Battistini, Jette L AU - Battistini JL AD - From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark. FAU - Svendsen, Kristina B AU - Svendsen KB AD - From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark. FAU - Sondergaard, Helle B AU - Sondergaard HB AD - From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark. FAU - Nilsson, Anna C AU - Nilsson AC AD - From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark. FAU - Illes, Zsolt AU - Illes Z AUID- ORCID: 0000-0001-9655-0450 AD - From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark. LA - eng PT - Journal Article DEP - 20240206 PL - United States TA - Neurology JT - Neurology JID - 0401060 RN - 0 (Aquaporin 4) RN - 0 (Antibodies) RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Neuromyelitis Optica/diagnosis MH - Aquaporin 4 MH - Antibodies MH - *Multiple Sclerosis/complications MH - Denmark/epidemiology MH - Autoantibodies EDAT- 2024/03/28 12:51 MHDA- 2024/03/29 06:46 CRDT- 2024/03/28 08:43 PHST- 2024/03/29 06:46 [medline] PHST- 2024/03/28 12:51 [pubmed] PHST- 2024/03/28 08:43 [entrez] AID - 10.1212/WNL.0000000000209147 [doi] PST - ppublish SO - Neurology. 2024 Mar 12;102(5):e209147. doi: 10.1212/WNL.0000000000209147. Epub 2024 Feb 6.