The mechanism and clinical features of paraneoplastic retinopathy

Authors:Yang Lizhu,  Sui Ruifang
DOI: 10.3760/cma.j.issn.2095-0160.2016.05.018
Published 2016-05-10
Cite as Chin J Exp Ophthalmol, 2016,34(5): 476-480.

Abstract

Paraneoplastic retinopathy (PR) is a rare form of paraneoplastic syndrome, it occurs when autoantibodies of neoplasms cross-react with autoantigens in normal retina.PR includes carcinoma-associated retinopathy (CAR), melanoma-associated retinopathy (MAR) and bilateral diffuse uveal melanocytic proliferation (BDUMP). Many kinds of autoantigens are involved in the process of CAR, like recoverin, α-enolase, α-transducin and so on.TRPM1 is a component of an ion channel of bipolar cell, it plays an important role in MAR.There are fewer kinds of antibodies associated with BDUMP, only anti-recoverin and anti-heat shock protein 70 (HSP70) antibodies were found in one particular research.The clinical manifestation of PR includes rapid vision loss of both eyes, photopsia and nyctalopia.The fundus can be normal or mild abnormal.Electroretinogram (ERG) presents various abnormalities based on the type of PR and autoantibody.The treatment of PR mainly targets on the original tumor, meanwhile glucocoticoids, plasmapheresis and intravenous immune globulin are also applied singly or combinedly in clinic.

Key words:

Paraneoplastic syndrome; Retinal disease; Autoimmunity

Contributor Information

Yang Lizhu
Department of Ophthalmology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing100730, China
Sui Ruifang
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Updated: October 9, 2019 — 3:04 am