Non-paraneoplastic related retinopathy: clinical challenges and review. (4th July 2019)

• Record Type: Journal Article
• Authors: Takiuti, Júlia T.; Takahashi, Vitor K. L.; Xu, Christine L.; Jauregui, Ruben; Tsang, Stephen H.
• Is Part Of: Ophthalmic genetics. Volume 40:Number 4(2019)
• Page Start: 293
• Page End: 297
• Publication Date: 2019-07-04
• Subjects: Anti-retinal antibodies -- autoimmune retinopathy -- immunosuppressant -- retinal antigens -- treatment; Eye -- Diseases -- Genetic aspects -- Periodicals; Eye Diseases -- genetics -- Periodicals; Eye Diseases -- in infancy & childhood -- Periodicals; 617.7
• DOI: 10.1080/13816810.2019.1650072
• Languages: English
• ISSNs: 1381-6810

Abstract

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.