ABSTRACT
Purpose: Placoid-pigment-epitheliopathies like Acute-Posterior-Multifocal-Placoid-Pigment Epitheliopathy (APMPPE) and Relentless-Placoid-Choroidopathy (RPC) usually show bilateral involvement. This retrospective case series describes the clinical features with unilateral disease onset.
Methods: 21 patients from 9 tertiary referring institutions were collected and evaluated.
Results: Seventeen patients were included: 11 (65%) had unilateral placoid-pigment-epitheliopathy consistent with APMPPE, 6 (35%) with RPC (mean age: 28.82 ± 9.1 years). Mean follow-up time was 45 ± 48 months. Forty-one percent fellow-eyes had initial inflammatory signs including vitreous-cells, vascular-sheathing, and ONH swelling. Thirty-five percent developed chorioretinal lesions during follow-up after 19.5 ± 16.9 months. Initial inflammation in fellow-eye was not associated with fellow-eye involvement (p = .5). However, subfoveal choroidal-thickness (SCT) at baseline was significantly thicker in fellow-eyes who developed chorioretinal lesion compared to fellow-eyes who did not (400 ± 70 µm versus 283 ± 127 µm,p = .047).
Conclusion: Unaffected fellow-eyes may have inflammatory signs at initial disease-onset and may develop lesions over time. They should be carefully monitored, particularly if they present with an increased SCT.
Acknowledgments
We thank Danielle Cuenoud for the English editing of the manuscript.
Financial disclosure
Marion R Munk is a consultant for Zeiss, Lumithera, GenSight Biologics, Novartis and Bayer. Jay Chhablani is a consultant for Allergan, Bayer, and Novartis. Martin S. Zinkernagel is a consultant for Bayer (C), Novartis (F, C), Heidelberg Engineering (N), Allergan (C). Albert T. Vitale is a consultant for AbbVie and ACIONT. The remaining authors have no financial interests or conflict of interests to disclose.