The enigma of autoimmune retinopathy.

Abstract

Autoimmune retinopathy (AIR) refers to an immunologic process whereby retinal antigens are aberrantly recognized as autoantigens, leading to retinal degeneration. AIR was initially described in 1976 as a paraneoplastic syndrome, termed cancer-associated retinopathy (CAR), secondary to a remote malignancy.1 Laboratory investigations revealed that the serum of patients with CAR contained autoantibodies against the photoreceptor protein recoverin, which are believed to represent the underlying etiology of CAR.2,3 A related syndrome, termed melanoma-associated retinopathy (MAR), was reported in patients with cutaneous malignant melanoma who were found to have autoantibodies to an unknown antigen on bipolar cells.4,5 Since that time, AIR in the absence of neoplasm, termed nonparaneoplastic AIR (npAIR), has been described as well.6 Along with this has come the identification of several novel putative antiretinal autoantibodies. Although all these novel discoveries have expanded our knowledge of AIR, they have also brought with them some confusion. There are numerous excellent reviews of AIR that have recently been published in the literature.7–13 The purpose of this current article is not to provide another review of this topic, but rather to highlight some of the ambiguity and uncertainty that exists in this field. For the purposes of this review, the term AIR will be used to refer to CAR, npAIR, and MAR, as the ocular features and proposed pathogenesis of these entities are essentially the same.