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Clinical and multimodal imaging features of uveitis and retinopathy associated with immune checkpoint inhibitors and targeted therapies

To describe multimodal imaging of Vogt-Koyanagi-Harada disease (VKHD)-like panuveitis associated with immune checkpoint inhibitors (ICIs) and targeted therapies (BRAF inhibitors) in patients with advanced or metastatic melanoma.

Inclusion criteria were patients with melanoma, treated with immunotherapy and/or BRAFi/MEKi, experiencing ocular symptoms during treatment (red eye, pain or blurred vision) with a diagnosis of uveitis. Patients’ evaluation included a dermatological medical history and a complete ophthalmologic examination. The examination included the best-corrected visual acuity, slit-lamp biomicroscopy, a retinal examination and spectral-domain optical coherence tomography. Angiography and swept-source OCT with measurement of the subfoveal choroidal thickness were performed in some patients.

Eight eyes of four patients with VKHD-like were evaluated. VKHD-like patients displayed choroidal thickening, choroidal folds associated with SRF on SD-OCT. FA and ICGA were performed in 2 patients. FA did not show significant delayed choroidal perfusion. In the late phase multiple hyperfluorescent pinpoints occurred around the optic disc with late peripapillary staining and disc hyperfluorescence. Hypofluorescent radial streaks corresponded to choroidal folds. ICGA showed twirled and hyperfluorescent segments of choroidal vessels in the early phase and the progressive apparition of some hyperfluorescent points, less numerous than on FA. On the mid phase of ICGA the choroidal folds were well visible, as well as many hypofluorescent small dots.

Ocular IrAEs are rare and are found in 1-3 % of patients treated by ICI. Most of the time they manifest as uveitis and dry eye. Several case reports have described type of panuveitis similar to VKHD. In our study we decribed multimodal imaging of eight eyes of four patients with VKHD-like panuveitis. To our knowledge we report here the largest series of pseudo-VKHD panuveitis.

The recognition of VKHD-like panuveitis on imaging by ophthalmologists is important in order to introduce rapid treatment in agreement with oncologists