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 revie...

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Main Authors: Thomas A. Weppelmann MD, PhD, MPH, Sabrina Khalil MD, Nabeel Zafrullah MD, Sabah Amir MD, MBA, Curtis E. Margo MD, MPH
Format: Article
Language:English
Published: SAGE Publishing 2022-12-01
Series:Cancer Control
Online Access:https://doi.org/10.1177/10732748221144458
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author Thomas A. Weppelmann MD, PhD, MPH
Sabrina Khalil MD
Nabeel Zafrullah MD
Sabah Amir MD, MBA
Curtis E. Margo MD, MPH
author_facet Thomas A. Weppelmann MD, PhD, MPH
Sabrina Khalil MD
Nabeel Zafrullah MD
Sabah Amir MD, MBA
Curtis E. Margo MD, MPH
author_sort Thomas A. Weppelmann MD, PhD, MPH
collection DOAJ
description 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.
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spelling doaj.art-4dcb6373b30d464ba0d35517bed8515b2023-04-27T04:03:29ZengSAGE PublishingCancer Control1526-23592022-12-012910.1177/10732748221144458Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune RetinopathyThomas A. Weppelmann MD, PhD, MPHSabrina Khalil MDNabeel Zafrullah MDSabah Amir MD, MBACurtis E. Margo MD, MPHBackground: 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.https://doi.org/10.1177/10732748221144458
spellingShingle Thomas A. Weppelmann MD, PhD, MPH
Sabrina Khalil MD
Nabeel Zafrullah MD
Sabah Amir MD, MBA
Curtis E. Margo MD, MPH
Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune Retinopathy
Cancer Control
title Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune Retinopathy
title_full Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune Retinopathy
title_fullStr Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune Retinopathy
title_full_unstemmed Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune Retinopathy
title_short Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune Retinopathy
title_sort ocular paraneoplastic syndromes a critical review of diffuse uveal melanocytic proliferation and autoimmune retinopathy
url https://doi.org/10.1177/10732748221144458
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