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Review
. 2019 Aug;40(4):293-297.
doi: 10.1080/13816810.2019.1650072. Epub 2019 Aug 8.

Non-paraneoplastic related retinopathy: clinical challenges and review

Júlia T Takiuti  1   2   3 , Vitor K L Takahashi  1   3   4 , Christine L Xu  1   3 , Ruben Jauregui  1   3   5 , Stephen H Tsang  1   3   6
Affiliations
Review

Non-paraneoplastic related retinopathy: clinical challenges and review

Júlia T Takiuti et al. Ophthalmic Genet. 2019 Aug.

Abstract

Autoimmune retinopathy (AIR) is a rare inflammatory condition characterized by progressive visual loss, abnormalities in visual fields and electroretinographic exams, along with presence of circulating anti-retinal antibodies. There are two main forms of AIR: paraneoplastic AIR (pAIR) and presumed non-paraneoplastic AIR (npAIR). NpAIR is considered a diagnosis of exclusion, since it is typically made after other causes of retinopathy have been investigated and the absence of malignancy is confirmed. Work-up of a npAIR case is challenging since there are no standartizaded protocols for diagnosis and treatment. The treatment regimen may vary from case to case, and it can be best guided by a set of parameters including electrophysiological responses, visual outcomes, and presence of anti-retinal antibodies. The purpose of this review is to summarize the principal clinical features, investigation, and management of npAIR.

Keywords: Anti-retinal antibodies; autoimmune retinopathy; immunosuppressant; retinal antigens; treatment.

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Conflict of interest statement

Financial and competing interests disclosure: The authors declare no conflicts of interest.

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