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Original research
Neuromyelitis optica spectrum disorder: pregnancy-related attack and predictive risk factors
  1. Liang Wang1,
  2. Lei Zhou1,
  3. Jingzi ZhangBao1,
  4. Wenjuan Huang1,
  5. Xuechun Chang1,
  6. Chuanzhen Lu1,
  7. Min Wang2,
  8. Wenyu Li3,
  9. Junhui Xia4,
  10. Xiang Li4,
  11. Lilin Chen5,
  12. Wei Qiu6,
  13. Jiahong Lu1,
  14. Chongbo Zhao1,
  15. Chao Quan1
  1. 1Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China
  2. 2Department of Ophthalmology and Vision Science, Eye Ear Nose and Throat Hospital, Shanghai Medical College, Fudan University, Shanghai, China
  3. 3Department of Neurology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
  4. 4Department of Neurology, First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
  5. 5Xiuquan Community Health Service Center, Guangzhou, Guangdong, China
  6. 6Department of Neurology, Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China
  1. Correspondence to Professor Chao Quan, Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai 200040, China; chao_quan{at}


Objectives To investigate the influence of pregnancy on patients with neuromyelitis optica spectrum disorder (NMOSD) and to identify risk factors that predict pregnancy-related attack.

Methods From January 2015 to April 2019, 418 female patients with NMOSD were registered at Huashan Hospital. We retrospectively reviewed their medical records and identified 110 patients with 136 informative pregnancies, of whom 83 were aquaporin-4 antibody (AQP4-ab)-positive and 21 were myelin oligodendrocyte glycoprotein-antibody-positive. Pregnancy-related attack was defined as an attack that occurred during pregnancy or within 1 year after delivery/abortion. We compared annualised relapse rate (ARR) during 12 months before pregnancy with that during every trimester of pregnancy and after delivery/abortion. Multivariate analyses were used to explore the independent risk factors involved and a nomogram was generated for the prediction of pregnancy-related attack. Thirty-five female patients from 3 other centres formed an external cohort to validate this nomogram.

Results ARR increased significantly during the first trimester after delivery (p<0.001) or abortion (p=0.019) compared with that before pregnancy. Independent risk factors predicting pregnancy-related attack included age at delivery/abortion (20–26.5, p=0.018; 26.5–33, p=0.001), AQP4-ab titre (≥1:100, p=0.049) and inadequate treatment during pregnancy and postpartum period (p=0.004). The concordance index of nomogram was 0.87 and 0.77 using bootstrap resampling in internal and external validation.

Conclusions The first trimester post partum is a high-risk period for NMOSD recurrence. Patients with younger age, higher AQP4-ab titre and inadequate treatment are at higher risk for pregnancy-related attack.

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  • Contributors LW designed and conceptualised the study, interpreted and analysed the data and drafted and revised the manuscript for intellectual content. LZ, JZ, WH and XC played a major role in the acquisition of data and revised the manuscript for intellectual content. CL and MW revised the manuscript for intellectual content. WL, JX, XL, LC and WQ played a major role in the acquisition of data of the validation cohort and contributed on data interpretation. WQ, JL and CZ revised the manuscript for intellectual content. CQ designed and conceptualised the study, interpreted and analysed the data and revised the manuscript for intellectual content. The corresponding author had full access to all the data in the study and had final responsibility for the decision to submit for publication.

  • Funding This research was supported by the National Natural Science Foundation of China (grant no. 81771296, 81801196), the Shanghai Municipal Science and Technology Major Project (grant no. 2018SHZDZX01) and ZHANGJIANG LAB, and the National Key Research and Development Program of China (grant no. 2016YFC0901504).

  • Competing interests LW, LZ, JZ, WH, XC, CL, MW, WL, JX, XL, LC, WQ, JL, CZ and CQ are sponsor of the current study and report no disclosures.

  • Patient consent for publication Not required.

  • Ethics approval The study was approved by the Medical Ethics Committee of Huashan Hospital, Fudan University (HIRB-2020007). Written informed consent was obtained from each patient.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data are available on reasonable request. Anonymised data not exhibited in our study will be made available on request from any qualified investigator.

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