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Long-term outcome of a Chinese cohort idiopathic retinitis, vasculitis, aneurysms, and neuroretinits (IRVAN) patients

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Abstract

Purpose

To evaluate the clinical characteristics and treatment outcomes of idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN) in a cohort of Chinese patients.

Materials

The clinical history, imaging evaluation, treatment and outcomes of 42 eyes in 21 patients diagnosed with IRVAN in a 15-year period were reviewed.

Results

Most patients were females (90%) ranged from 15 to 58 years old. The initial decimal best corrected visual acuity (BCVA) of the patients ranged from light perception (LP) to 1.5 (0.55 ± 0.38). Eighteen eyes were in stage 2; 21 eyes in stage 3; and 1 in stage 5 at the initial visits according to the present staging system. Two eyes had vitreoretinal fibrovascular proliferation (FVP) and tractional retinal detachment (RD) at the initial visit. Intra-retinal microvascular abnormality (IRMA) was found in 7 eyes. Thirty-four eyes received retinal photocoagulation, 27 of which were pan-retinal photocoagulation (PRP). Total of 8 PPV were performed for VH, vitreoretinal FVP and RD, and macular epimembrane. Aneurysms on the head of optic nerve and artery bifurcations disappeared in 8 eyes and decreased in number in 2 eyes 1 year after photocoagulation. However, the BCVA of the patients did not have significant difference from that at the initial visits (P = 0.534). Seven eyes suffered severe visual impairment (BCVA ≤ 0.1) due to vitreoretinal FVP and tractional RD (3), exudative maculopathy (2), paracentral acute middle maculopathy (PAMM)(1), and neovascular glaucoma (NVG) (1).

Conclusions

We found that IRVAN have a predilection to female gender. Vitreoretinal FVP and tractional RD and exudative maculopathy are major causes of severe visual impairment in IRVAN patients. We propose to revise the present staging system to include vitreoretinal FVP and RD in the staging of IRVAN patients.

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Availability of data and materials

Transcripts of this study are available from the corresponding author on reasonable request; however, all the identifying and confidential information of the participants would be removed.

Abbreviations

BCVA:

Best corrected visual acuity

FFA:

Fundus fluorescein angiography

IRVAN:

Idiopathic retinitis, vasculitis, aneurysms, and neuroretinits

IRMA:

Intra-retinal microvascular abnormality

NVG:

Neovascular glaucoma

OCT:

Optical coherence tomography

PPV:

Pars plana vitrectomy

PRP:

Pan-retinal photocoagulation

FVP:

Fibrovascular proliferation

RD:

Retinal detachment

VH:

Vitreous hemorrhage

PAMM:

Paracentral acute middle maculopathy

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Acknowledgements

The authors would like to acknowledge all of the participants and staff for their contributions to this research.

Funding

This work was supported by Grant from the Science and Technology Commission of Shanghai Municipality (No. SHDC12016116). The funding organization had no role in the design and conduct of this research.

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Author notes

  1. Yongjin Zhang and Gezhi Xu contributed equally as corresponding authors.

Authors and Affiliations

  1. Eye Institute and Department of Ophthalmology, Eye and ENT Hospital, Fudan University, 83 Fenyang Rd, Shanghai, 200031, China

    Yuhe Qi, Yongjin Zhang, Wei Liu, Lei Li, Xiaofeng Ye, Qing Chang, Min Wang, Yi Xuan, Jingli Guo & Gezhi Xu

  2. NHC Key Laboratory of Myopia (Fudan University); Key Laboratory of Myopia, Chinese Academy of Medical Sciences, Shanghai, China

    Yuhe Qi, Yongjin Zhang, Wei Liu, Lei Li, Xiaofeng Ye, Qing Chang, Min Wang, Yi Xuan, Jingli Guo & Gezhi Xu

  3. Shanghai Key Laboratory of Visual Impairment and Restoration, Shanghai, China

    Yuhe Qi, Yongjin Zhang, Wei Liu, Lei Li, Xiaofeng Ye, Qing Chang, Min Wang, Yi Xuan, Jingli Guo & Gezhi Xu

Authors
  1. Yuhe Qi
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  2. Yongjin Zhang
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  3. Wei Liu
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  4. Lei Li
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  6. Qing Chang
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  8. Yi Xuan
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  10. Gezhi Xu
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Contributions

QY and ZY designed, analyzed, interpreted the patients’ data and wrote the manuscript; ZY and XG reviewed the manuscript; LW, LL, YX, CQ, WM, XY and GJ collected the patients’ data. ZY, XG, LW, LL, YX and CQ performed the medical treatment including laser photocoagulation and pars plana vitrectomy. All the authors read and approved the final manuscript.

Corresponding author

Correspondence to Yongjin Zhang.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethics approval

This study was approved by the Institutional Review Board of the Eye and ENT Hospital of Fudan University. The principles of the Declaration of Helsinki were followed in all procedures.

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Written informed consent was obtained from all participants or their guardians.

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Qi, Y., Zhang, Y., Liu, W. et al. Long-term outcome of a Chinese cohort idiopathic retinitis, vasculitis, aneurysms, and neuroretinits (IRVAN) patients. Int Ophthalmol 41, 3487–3496 (2021). https://doi.org/10.1007/s10792-021-01915-w

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  • DOI: https://doi.org/10.1007/s10792-021-01915-w

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