Immune checkpoint inhibitor-associated Vogt-Koyanagi-Harada-like syndrome: A descriptive systematic review

Abstract Topic Vogt-Koyanagi-Harada (VKH)-like uveitis is uniquely reported with immune checkpoint inhibitors (ICI) and BRAF/MEK inhibitors. This article aims to provide a comprehensive portrait of the comorbidities, ocular presentations, treatments, and visual outcomes of patients with VKH-like uve...

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Main Authors: Huixin Anna Zhang, Amelia T. Yuan, Noémie Chiasson, Kevin Y. Wu, Ananda Kalevar
Format: Article
Language:English
Published: SpringerOpen 2025-05-01
Series:Journal of Ophthalmic Inflammation and Infection
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Online Access:https://doi.org/10.1186/s12348-025-00484-8
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author Huixin Anna Zhang
Amelia T. Yuan
Noémie Chiasson
Kevin Y. Wu
Ananda Kalevar
author_facet Huixin Anna Zhang
Amelia T. Yuan
Noémie Chiasson
Kevin Y. Wu
Ananda Kalevar
author_sort Huixin Anna Zhang
collection DOAJ
description Abstract Topic Vogt-Koyanagi-Harada (VKH)-like uveitis is uniquely reported with immune checkpoint inhibitors (ICI) and BRAF/MEK inhibitors. This article aims to provide a comprehensive portrait of the comorbidities, ocular presentations, treatments, and visual outcomes of patients with VKH-like uveitis following ICI therapy. Clinical relevance ICIs are increasingly used in cancer therapy, but poorly understood ocular immune-related adverse events (irAEs) can lead to suspension of treatment and be vision-threatening. Methods We conducted a systematic review (PROSPERO #CRD42024558269) according to PRISMA guidelines. MEDLINE, Embase, CENTRAL, and Web of Science were searched for English articles published up to June 28, 2024. All study designs reporting on incident VKH-like uveitis following ICI were included. Risk of Bias was assessed using a tool modified from Murad et al. (2018). Results Of 865 articles, we included 42 articles (4 observational studies, 28 case reports, 6 case series, 3 letters, and 1 editorial) from 12 countries, comprising 52 patients. The mean age was 60.0 ± 11.9 years, and 32 (61.5%) were females. Thirty-six (69.2%) had melanoma, and most were undergoing treatment with a PD-1 inhibitor alone (n = 33, 63.5%) or in combination with a CTLA-4 inhibitor (n = 10, 19.2%). The mean duration of ICI treatment before VKH-like uveitis symptoms was 22.2 ± 29.6 weeks, and the mean duration of ocular symptoms was 16.7 ± 18.6 weeks, with wide variation. Overall, 43 patients (73.1%) had imaging or exams suggesting bilateral involvement and 21 cases (40.4%) suggesting panuveitis. Only 31 cases (59.6%) met the acute initial-onset uveitis criteria, and 15 (28.8%) met the chronic phase criteria. Most (n = 47, 90.4%) required systemic or intravitreal steroids, termination of ICI (n = 31, 59.6%), and experienced full resolution or remission of visual symptoms (n = 43, 82.7%). Most articles (n = 40, 95.2%) were judged to be at medium risk of bias. Conclusion This descriptive systematic review consisted mostly of case reports, but it confirmed that a high proportion of VKH-like uveitis occur with PD-1 inhibitors and melanoma patients. VKH-like uveitis can lead to suspension of treatment. Further collaboration between oncologists and ophthalmologists is needed in the continuum of cancer care.
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spelling doaj-art-1fb0356a4f8f4b89b7669e2de8f2b3382025-08-20T02:30:43ZengSpringerOpenJournal of Ophthalmic Inflammation and Infection1869-57602025-05-0115112410.1186/s12348-025-00484-8Immune checkpoint inhibitor-associated Vogt-Koyanagi-Harada-like syndrome: A descriptive systematic reviewHuixin Anna Zhang0Amelia T. Yuan1Noémie Chiasson2Kevin Y. Wu3Ananda Kalevar4Department of Ophthalmology and Vision Sciences, University of TorontoSchulich School of Medicine and Dentistry, University of Western OntarioDepartment of Medicine, Université de SherbrookeFaculty of Medicine, Université LavalDepartment of Surgery, Division of Ophthalmology, University of SherbrookeAbstract Topic Vogt-Koyanagi-Harada (VKH)-like uveitis is uniquely reported with immune checkpoint inhibitors (ICI) and BRAF/MEK inhibitors. This article aims to provide a comprehensive portrait of the comorbidities, ocular presentations, treatments, and visual outcomes of patients with VKH-like uveitis following ICI therapy. Clinical relevance ICIs are increasingly used in cancer therapy, but poorly understood ocular immune-related adverse events (irAEs) can lead to suspension of treatment and be vision-threatening. Methods We conducted a systematic review (PROSPERO #CRD42024558269) according to PRISMA guidelines. MEDLINE, Embase, CENTRAL, and Web of Science were searched for English articles published up to June 28, 2024. All study designs reporting on incident VKH-like uveitis following ICI were included. Risk of Bias was assessed using a tool modified from Murad et al. (2018). Results Of 865 articles, we included 42 articles (4 observational studies, 28 case reports, 6 case series, 3 letters, and 1 editorial) from 12 countries, comprising 52 patients. The mean age was 60.0 ± 11.9 years, and 32 (61.5%) were females. Thirty-six (69.2%) had melanoma, and most were undergoing treatment with a PD-1 inhibitor alone (n = 33, 63.5%) or in combination with a CTLA-4 inhibitor (n = 10, 19.2%). The mean duration of ICI treatment before VKH-like uveitis symptoms was 22.2 ± 29.6 weeks, and the mean duration of ocular symptoms was 16.7 ± 18.6 weeks, with wide variation. Overall, 43 patients (73.1%) had imaging or exams suggesting bilateral involvement and 21 cases (40.4%) suggesting panuveitis. Only 31 cases (59.6%) met the acute initial-onset uveitis criteria, and 15 (28.8%) met the chronic phase criteria. Most (n = 47, 90.4%) required systemic or intravitreal steroids, termination of ICI (n = 31, 59.6%), and experienced full resolution or remission of visual symptoms (n = 43, 82.7%). Most articles (n = 40, 95.2%) were judged to be at medium risk of bias. Conclusion This descriptive systematic review consisted mostly of case reports, but it confirmed that a high proportion of VKH-like uveitis occur with PD-1 inhibitors and melanoma patients. VKH-like uveitis can lead to suspension of treatment. Further collaboration between oncologists and ophthalmologists is needed in the continuum of cancer care.https://doi.org/10.1186/s12348-025-00484-8Immune checkpoint inhibitorVogt-Koyanagi-Harada-like uveitisPanuveitisDrug-inducedImmune related adverse event
spellingShingle Huixin Anna Zhang
Amelia T. Yuan
Noémie Chiasson
Kevin Y. Wu
Ananda Kalevar
Immune checkpoint inhibitor-associated Vogt-Koyanagi-Harada-like syndrome: A descriptive systematic review
Journal of Ophthalmic Inflammation and Infection
Immune checkpoint inhibitor
Vogt-Koyanagi-Harada-like uveitis
Panuveitis
Drug-induced
Immune related adverse event
title Immune checkpoint inhibitor-associated Vogt-Koyanagi-Harada-like syndrome: A descriptive systematic review
title_full Immune checkpoint inhibitor-associated Vogt-Koyanagi-Harada-like syndrome: A descriptive systematic review
title_fullStr Immune checkpoint inhibitor-associated Vogt-Koyanagi-Harada-like syndrome: A descriptive systematic review
title_full_unstemmed Immune checkpoint inhibitor-associated Vogt-Koyanagi-Harada-like syndrome: A descriptive systematic review
title_short Immune checkpoint inhibitor-associated Vogt-Koyanagi-Harada-like syndrome: A descriptive systematic review
title_sort immune checkpoint inhibitor associated vogt koyanagi harada like syndrome a descriptive systematic review
topic Immune checkpoint inhibitor
Vogt-Koyanagi-Harada-like uveitis
Panuveitis
Drug-induced
Immune related adverse event
url https://doi.org/10.1186/s12348-025-00484-8
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