Paraneoplastic retinopathies (PR), including cancer-associated retinopathy (CAR) or the closely related melanoma-associated retinopathy (MAR) occur in a small subset of patients with retinal degeneration and systemic cancer. This autoimmune syndrome is characterized by sudden, progressive loss of vision in association with circulating anti-retinal autoantibodies. The PR syndromes are heterogeneous, may produce a number of ocular symptoms, and may be associated with several different neoplasms, including lung, breast, prostate, gynecological, and colon cancer, melanoma, and hematologic malignancies. We examined the onset of retinopathy in correlation to the diagnosis of cancer and the presence of specific anti-retinal autoantibodies in PR patients. In some patients without diagnosed malignant tumors, the onset of ocular symptoms and the presence of autoantibodies preceded the diagnosis of cancer by months to years, including anti-recoverin, antitransducin-α, and anti-carbonic anhydrase II antibodies. Although anti-retinal autoantibodies may not be a good predictor of a specific neoplasm, they can be used as biomarkers for different subtypes of retinopathy. Identification of autoantibodies involved in autoimmune-mediated PR will help elucidate the mechanisms underlying the PR syndromes and develop targeted therapies for these sight-threatening disorders.
KeywordsCancer-associated retinopathy; anti-retinal autoantibodies; recoverin; enolase; autoimmune retinopathy
Take home message• Retinal autoantigens can be used as biomarkers for different subtypes of CAR.• Autoantibodies to retinal antigens that also recognize tumor autoantigens may be useful biomarkers for the cause and origin of paraneoplastic disease.• In some patients, the onset of ocular symptoms and the detection of anti-retinal autoantibodies may precede the diagnosis of systemic cancer.• The recognition of retinopathy as part of a paraneoplastic syndrome should prompt an immediate search for an occult neoplasm.Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. • Early detection of anti-retinal autoantibodies may serve to identify those who are at risk of retinal deterioration and blindness.
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