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Bilateral central retinal artery occlusion as a presenting manifestation of systemic lupus erythematosus: a case-based review

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Abstract

Central retinal artery occlusion (CRAO) is an ophthalmic emergency that typically results in permanent vision damage even despite vigorous treatment. In this article, we describe a case of acute vaso-occlusive retinopathy that presented as the primary manifestation of SLE in the absence of elevated levels of APLAs. After treatment with intravenous steroids, immunoglobulin, intrathecal injection of dexamethasone, plasma exchange, and intravenous cyclophosphamide, SLE was well controlled in the patient, but her vision was permanently lost in the left eye. We also go over a brief review of the currently available literature on retinal vaso-occlusive disease present in SLE. The pathology mechanism of CRAO is related to immune complex-mediated “vasculitis”, which is typically associated with neuropsychiatric lupus. However, the literature review identified antiphospholipid antibody syndrome (APS) in only 6 of 19 patients, indicating that other mechanisms besides APS are associated with CRAO. Systemic immunosuppression and anticoagulants are required for the treatment of this severe vaso-occlusive retinopathy. Early recognition and aggressive intervention may prevent severe loss of vision.

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Acknowledgements

The authors thank the patient and her family for their cooperation, and Mrs Kailey Brodeur for editing support.

Funding

The Natural Science Foundation of Zhejiang Province (No. LY20H100007, LY22H100004, LY22H120003) and National Natural Science Foundation of China (No. 82271817) funded this study.

Author information

Author notes

  1. Xin Chen and Xin Chen, Jinyu Li authors equally contributed to this work and share first authorship.

Authors and Affiliations

  1. Department of Rheumatology, The Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China

    Xin Chen, Xiaowei Shi, Wenwen Wang, Qi Cheng, Yifan Xie, Jing Xue & Yan Du

  2. Department of Rheumatology and Immunology, Jinhua Municipal Central Hospital, Jinhua Hospital of Zhejiang University, Jinhua, China

    Xin Chen & Xiaowei Shi

  3. Eye Center, The Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China

    Jinyu Li

  4. Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China

    Chao Wang

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  1. Xin Chen
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Contributions

YD and JX contributed to the research design and drafted the manuscript. XC, XWS JYL, and WWW collected clinical data and assisted in writing the manuscript. CW collected the magnetic reasoning image data. QC and YFX contributed to revising the manuscript. All authors approved the submitted version of the manuscript and agreed to accept responsibility for all its aspects.

Corresponding authors

Correspondence to Jing Xue or Yan Du.

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Conflict of interest

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Ethical approval

The Ethics Committee of Second Affiliated Hospital of Zhejiang University School of Medicine reviewed and approved this study of a human participant (20230014). The patient provided written informed consent to participate in this study and for the publication of any potentially identifiable images or data included in this article.

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Chen, X., Shi, X., Li, J. et al. Bilateral central retinal artery occlusion as a presenting manifestation of systemic lupus erythematosus: a case-based review. Rheumatol Int 43, 1947–1956 (2023). https://doi.org/10.1007/s00296-023-05365-8

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  • DOI: https://doi.org/10.1007/s00296-023-05365-8

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