Neuromyelitis optica spectrum disorders in Arabian Gulf (NMOAG); establishment and initial characterization of a patient registry.
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Shosha Eslam, Al Asmi Abdulla, Nasim Eman, Inshasi Jihad, Abdulla Fatima, Al Malik Yaser, Althobaiti Ahmed, Alzawahmah Mohamed, Alnajashi Hind A, Binfalah Mohamed, AlHarbi Awad, Thubaiti Ibtisam A, Ahmed Samar F, Al-Hashel Jasem, Elyas Mortada, Nandhagopal Ramachandiran, Gujjar Arunodaya, Harbi Talal Al, Towaijri Ghadah Al, Alsharooqi Isa A, AlMaawi Ahmed, Al Khathaami Ali M, Alotaibi Naser, Nahrir Shahpar, Al Rasheed Abdulrahman A, Al Qahtani Mohammed, Alawi Sadaga, Hundallah Khalid, Jumah Mohammed, Alroughani Raed, with the Guthy-Jackson Charitable Foundation International Clinical Consortium
Аннотация
METHODS: Retrospective multicentre study of hospital records of patients diagnosed with NMOSD based on 2015 International Panel on NMOSD Diagnosis (IPND) consensus criteria.
RESULTS: One hundred forty four patients were evaluated, 64.3% were anti-AQP4 antibody positive. Mean age at onset and disease duration were 31±12 and 7 ± 6 years respectively. Patients were predominantly female (4.7:1). Overall; relapsing course (80%) was more common than monophasic (20%). Optic neuritis was the most frequent presentation (48.6%), regardless of serostatus. The proportion of patients (54.3%) with visual acuity of ≤ 0.1 was higher in the seropositive group (p = 0.018). Primary presenting symptoms of transverse myelitis (TM) were observed in 29% of patients, and were the most significant correlate of hospitalization (p<0.001). Relative to anti-APQ4 serostatus, there were no significant differences in terms of age of onset, course, relapse rates or efficacy outcomes except for oligoclonal bands (OCB), which were more often present in seronegative patients (40% vs.22.5%; p = 0.054). Irrespective of serostatus, several disease modifying therapies were instituted including steroids or immunosuppressives, mostly, rituximab and azathioprine in the cohort irrespective of serostatus. The use of rituximab resulted in reduction in disease activity.
CONCLUSION: This is the first descriptive NMOSD cohort in the Arabian Gulf region. Seropositive patients were more prevalent with female predominance. Relapsing course was more common than monophasic. However, anti-AQP4 serostatus did not impact disease duration, relapse rate or therapeutic effectiveness. These findings offer new insights into natural history of NMOSD in patients of the Arabian Gulf and allow comparison with patient populations in different World regions.