Purpose: To review the current state for diagnosis and management of autoimmune retinopathy.
Methods: A review of the literature was performed, encompassing autoimmune retinopathy including paraneoplastic retinopathy (cancer-associated retinopathy, melanoma-associated retinopathy, and bilateral diffuse uveal melanocytic proliferation) and non-paraneoplastic autoimmune retinopathy. Based on this review, current principles and techniques for diagnosis and the treatments reported for autoimmune retinopathy are discussed with the aim to clarify some of the confusion that exists regarding this complex entity.
Results: Autoimmune retinopathy encompasses a spectrum of retinal degeneration phenotypes. The clinical features, fundus characteristics, and electroretinogram findings for paraneoplastic and non-paraneoplastic retinopathy are reviewed. The different antiretinal antibodies reported in these entities are described. The diagnostic approaches for detecting these antiretinal antibodies and their limitations are covered. The treatments reported for autoimmune retinopathy and their outcomes are reviewed.
Conclusion: Among the myriad of antiretinal antibodies reported, challenges persist in determining which antibodies are pathogenic and which are benign and what factors cause antiretinal antibodies to become pathologic. There also remain difficulties in the detection and accurate measurement of antiretinal antibodies, and the response to therapeutic intervention in autoimmune retinopathy is variable.
This review covers autoimmune retinopathy describing paraneoplastic retinopathy (cancer-associated retinopathy, melanoma-associated retinopathy, and bilateral diffuse uveal melanocytic proliferation) and non-paraneoplastic autoimmune retinopathy. The diagnostic utility and methods for detecting antiretinal antibodies associated with autoimmune retinopathy are discussed. The various treatments published for autoimmune retinopathy and their reported outcomes are reviewed.
*Department of Ophthalmology, Northwestern University Feinberg School of Medicine, Chicago, Illinois; and
†Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, the Pangere Center for Hereditary Retinal Diseases, Chicago, Illinois.
Reprint requests: Lee M. Jampol, MD, Department of Ophthalmology, Northwestern University Feinberg School of Medicine, 645 North Michigan Avenue, Suite 440, Chicago, IL 60611; e-mail: firstname.lastname@example.org
Supported in part by an unrestricted grant to the Northwestern Department of Ophthalmology from Research to Prevent Blindness (RPB, NY).
None of the authors have any conflicting interests to disclose.