Abstract

BackgroundNeuromyelitis Optica Spectrum Disorder (NMOSD) is an inflammatory disease of the central nervous system with preferential involvement of the optic nerve and spinal cord. However, data on NMOSD incidences in China, a country encompassing 20% of the world's population and covering vast areas of Eastern Asia, are unknown. MethodsWe conducted the first nationwide survey of NMOSD, based on the database of the National Hospital Quality Monitoring System (HQMS) of China, which covers all 1665 tertiary hospitals. The “Medical Record Homepage” of all patients were consistently collected via a standard protocol across each tertiary hospital. The primary outcome was the incidence of NMOSD, diagnosed according to the 2015 International Panel for Neuromyelitis Optica Diagnosis criteria and identified by ICD-10 code (G36•0). Burden of hospitalization, comorbidities, and death were also evaluated. FindingsWe identified 33,489 hospital admissions for 17,416 NMOSD diagnosed patients from 2016 to 2018. 11,973 patients were newly diagnosed NMOSD. The age and sex adjusted incidence per 100,000 person years was 0•278 (95% confidence interval [CI], 0•273–0•283), with 0•075 (0•069–0•08) in child and 0•347 (0•34–0•353) in adult. The peak age of onset NMOSD is 45–65 years with an incidence 0•445/100,000 (95% CI, 0•433–0•457). The female to male ratio was 4•71 (p<0•001, 95% CI, 4•50–4•94). Geographical distribution of NMOSD is not related to the latitude gradient. Sjögren's syndrome (1,124/17,416, 6•5%) and systemic lupus erythematosus (387/17,416, 2•2%) are the most frequently autoimmune comorbidities. 106 adults and 4 children of the 17,416 NMOSD cohort died. InterpretationThe incidence of NMOSD in China per 100,000 person years was 0•278, with 0•075 in child and 0•347 in adult. The geographical distribution of NMOSD is not related to the latitude gradient. FundingNational Science Foundation of China (91949208, 91642205, and 81830038); Advanced Innovation Center for Human Brain Protection, Capital Medical University, Beijing.

Highlights

  • Neuromyelitis optica spectrum disorder (NMOSD) is a severe inflammatory disease of the central nerves system (CNS) with preferential involvement of optic nerve and spinal cord [1]

  • We identified 33,489 hospital admissions for 17,416 patients of NMOSD from 2016 to 2018

  • The age and sex adjusted incidence was 0287 patients per 100,000 individuals, which is higher in adults than that of children (0347 versus 0075). This finding is consistent with the reported crude annual incidence of NMOSD in Malaysia (039 per 100,000), where 469% of prevalent cases are Chinese [22, 23]

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Summary

Introduction

Neuromyelitis optica spectrum disorder (NMOSD) is a severe inflammatory disease of the central nerves system (CNS) with preferential involvement of optic nerve and spinal cord [1]. Methods: We conducted the first nationwide survey of NMOSD, based on the database of the National Hospital Quality Monitoring System (HQMS) of China, which covers all 1665 tertiary hospitals. The primary outcome was the incidence of NMOSD, diagnosed according to the 2015 International Panel for Neuromyelitis Optica Diagnosis criteria and identified by ICD-10 code (G360). Findings: We identified 33,489 hospital admissions for 17,416 NMOSD diagnosed patients from 2016 to 2018. The age and sex adjusted incidence per 100,000 person years was 0278 (95% confidence interval [CI], 0273–0283), with 0075 (0069–008) in child and 0347 (034–0353) in adult. The peak age of onset NMOSD is 45–65 years with an incidence 0445/100,000 (95% CI, 0433–0457). Interpretation: The incidence of NMOSD in China per 100,000 person years was 0278, with 0075 in child and 0347 in adult.

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