Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune Retinopathy

Background: Dozens of paraneoplastic syndromes affect the visual system ranging from conjunctival pemphigoid to encephalopathy of the occipital cortex. The most profiled ocular syndromes are bilateral diffuse uveal melanocytic proliferation (BDUMP) and the autoimmune retinopathies.Purpose: To review the critical features of these 2 entities then concentrate on advancements in treatment made within the last 10 years.Study Design: Literature review with structured data abstraction.Results: Major insights into pathogenesis have been wanting. Plasmapheresis appears to improve vision in a substantial proportion of patients with BDUMP. The number of clinical variables that influence visual outcome in paraneoplastic retinopathies combined with the variety of local and systemic treatment options makes interpretation of clinical effectiveness difficult.Conclusions: The rarity of these disorders makes randomized clinical trials unlikely. It may be time for a clinical professional organization to use a modified Delphi method to establish a consensus algorithm for the diagnosis and management of retinal paraneoplastic syndromes to augment clinical communications and clinical trials.

Automated summary

This article reviews the critical features of bilateral diffuse uveal melanocytic proliferation and autoimmune retinopathies, as well as the recent advancements in treatment. The lack of understanding in pathogenesis and the challenges in evaluating clinical effectiveness are highlighted. The authors suggest establishing a consensus algorithm to improve clinical communications and trials.