BACKGROUND: Aquaporin-4-IgG positive (AQP4-IgG+) Neuromyelitis Optica Spectrum Disorder (NMOSD) is an uncommon central nervous system autoimmune disorder. Disease outcomes in AQP4-IgG+NMOSD are typically measured by relapse rate and disability. Using the MSBase, a multi-centre international registry, we aimed to examine the impact immunosuppressive therapies and patient characteristics as predictors of disease outcome measures in AQP4-IgG+NMOSD. METHOD: This MSBase cohort study of AQP4-IgG+NMOSD patients examined modifiers of relapse in a multivariable proportional hazards model and expanded disability status score (EDSS) using a mixed effects model. RESULTS: 206 AQP4-IgG+ patients were included (median follow-up 3.7 years). Age (hazard ratio [HR] = 0.82 per decade, p = 0.001), brainstem onset (HR = 0.45, p = 0.009), azathioprine (HR = 0.46, p<0.001) and mycophenolate mofetil (HR = 0.09, p = 0.012) were associated with a reduced risk of relapse. A greater EDSS was associated with age (β = 0.45 (per decade), p<0.001) and disease duration (β = 0.07 per year, p<0.001). A slower increase in EDSS was associated with azathioprine (β = -0.48, p<0.001), mycophenolate mofetil (β = -0.69, p = 0.04) and rituximab (β = -0.35, p = 0.024). INTERPRETATION: This study has demonstrated that azathioprine and mycophenolate mofetil reduce the risk of relapses and disability progression is modified by azathioprine, mycophenolate mofetil and rituximab. Age and disease duration were the only patient characteristics that modified the risk of relapse and disability in our cohort.

Bakirkoy Education and Research Hospital for Psychiatric and Neurological Diseases Istanbul Turkey

Bombay Hospital Institute of Medical Sciences Mumbai Maharashtra 400020 India

CORe Department of Medicine University of Melbourne Melbourne Australia; Department of Neurology Royal Melbourne Hospital Melbourne Australia; Department of Neurology Royal Melbourne Hospital Melbourne Australia

Department of Basic Medical Sciences Neuroscience and Sense Organs University of Bari Bari Italy

Department of Medicine and Surgery University of Parma via Gramsci 14 Parma 43126 Italy

Department of Neurology and Center of Clinical Neuroscience 1st Faculty of Medicine Charles University and General University Hospital Prague Czech Republic

Department of Neurology Faculty of Medicine University of Debrecen Moricz Zs krt 22 Debrecen 4032 Hungary

Department of Neurology Penang General Hospital Penang Malaysia

Department of Neurology Royal Brisbane and Women's Hospital Herston Brisbane 4029 Australia

Department of Neurology Royal Melbourne Hospital Melbourne Australia

Department of Neurology Westmead Hospital NSW 2145 Sydney Australia

Department of Neuroscience Azienda Ospedaliera Universitaria via giardini 1355 Modena 41100 Italy

Dipartimento di Scienze Biomediche e Neuromotorie Università di Bologna Italy

Division of Neurology Department of Medicine Amiri Hospital P O Box 1661 Qurtoba Sharq 73767 Kuwait

Dokuz Eylul University Cumhuriyet Blv 144 Konak Izmir 35220 Turkey

GF Ingrassia Department University of Catania Policlinico G Rodolico Via Santa Sofia 78 95123 Catania Italy

Hacettepe University Sihhiye Ankara 6100 Turkey

Haydarpasa Numune Training and Research Hospital Selimiye Mahallesi Istanbul 34668 Turkey

Hospital Universitario La Paz Madrid Spain

Hospital Universitario Virgen Macarena Dr Fedriani 3 Sevilla 41009 Spain

Instituto Vasco de Investigación Sanitaria Hospital UniversitarioDonostia Paseo de Begiristain San Sebastián 20014 Spain

IRCCS Istituto delle Scienze Neurologiche di Bologna University of Bologna Via Altura 3A 40139 Bologna Italy; Dipartimento di Scienze Biomediche e Neuromotorie Università di Bologna Italy

IRCCS Mondino Foundation via Mondino 2 Pavia 27100 Italy

Koc University School of Medicine Department of Neurology Davutpasa Istanbul Turkey

KTU Medical Faculty Farabi Hospital Karadeniz Technical University Trabzon 61080 Turkey

Medical Faculty 19 Mayis University Kurupelit Samsun 55160 Turkey

Monash University Department of Neurology Alfred Hospital Melbourne Australia

Nehme and Therese Tohme Multiple Sclerosis Center American University of Beirut Medical Center Beirut Lebanon

Neuro Rive Sud 4896 boul Taschereau suite 250 Quebec J4V 2J2 Canada

Neurology Department King Fahad Specialist Hospital Dammam Al Muraikebat Area Ammar Bin Thabet Street P O Box 15215 Dammam 31444 Saudi Arabia

New York University Langone Medical Center 240 East 38th St New York 10016 United States

School of Medicine and Public Health Department of Neurology University Newcastle Lookout Road John Hunter Hospital Hunter New England Health Newcastle 2305 Australia

Semmelweis University Budapest Balassa Budapest 1083 Hungary

Universidade Metropolitana de Santos Santos Brazil

Universitary Hospital Ghent Corneel Heymanslaan 10 9000 Ghent Belgium

UOC Neurologia Azienda Sanitaria Unica Regionale Marche AV3 Via Santa Lucia 2 Macerata 62100 Italy

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