Abstract
PurposeAutoimmune retinopathies and optic neuropathies are complex disorders of the retina and the optic nerve, in which patients develop autoantibodies (AAbs) against retinal and optic nerve proteins. Autoimmunity might significantly influence the outcome of retinal and optic nerve degenerative process but the pathogenic process is not fully elucidated. To better understand the role of AAbs in pathogenicity of these suspected autoimmune visual disorders, we focused on unique AAbs specificities associated with the syndrome to identify their antigenic targets in the optic nerve and retina.MethodsSerum samples were obtained from patients, whose visual disorders were potentially autoimmune in nature, including patients with cancer with possible paraneoplastic syndrome. Autoantibodies were tested against human optic nerve and retinal antigens for specificity by Western blotting and immunofluorescence.ResultsOut of 209 tested for anti-optic nerve autoantibodies, 55% showed specific neuronal autoantibodies. The repertoire of anti-optic nerve autoantibodies often differed from anti-retinal antibodies. The major antigenic targets for these antibodies could be divided into four groups. Autoantibodies specific to classical glycolytic enzymes involved in energy production (α and γ enolases, glyceraldehyde 3-phosphate dehydrogenase) also reacted with retinal antigens. Autoantibodies targeted neuronal-specific myelin proteins (MBP, MOG), aquaporin 4, and collapsing response mediator protein 5 reacted with optic nerve antigens. They showed immunostaining of axons and myelin in the optic nerve as determined by double immunofluorescence.ConclusionWe identified novel neuronal autoantigens not previously known to be associated with acquired autoimmune retinopathy and optic neuropathy. Knowledge of the full autoantibody repertoire perpetuating this syndrome is an important first requirement in increasing our understanding of the autoimmune process to facilitate better diagnosis, prognosis, and treatment.
Highlights
Presence of autoantibodies (AAbs) specific against antigens present in the retina is the hallmark of autoimmune process in cancer-associated retinopathy (CAR) and autoimmune retinopathy (AR) [1, 2]
Autoantibodies specific to classical glycolytic enzymes involved in energy production (α and γ enolases, glyceraldehyde 3-phosphate dehydrogenase) reacted with retinal antigens
Autoantibodies targeted neuronal-specific myelin proteins (MBP, myelin oligodendrocyte glycoprotein (MOG)), aquaporin 4, and collapsing response mediator protein 5 reacted with optic nerve antigens
Summary
Presence of autoantibodies (AAbs) specific against antigens present in the retina is the hallmark of autoimmune process in cancer-associated retinopathy (CAR) and autoimmune retinopathy (AR) [1, 2]. Because there is little known about pathogenicity of retinopathy and associated optic neuropathy, we undertook a first line of investigation focusing on unique AAbs specificities associated with the syndrome that is thought to be autoimmune in nature, including patients with cancer with potential paraneoplastic syndrome. These patients had symptoms of optic nerve and/or retinal dysfunction, some of which have been found to have electrophysiological evidence of retinal process, or other findings to suggest an optic nerve process. Our study shows that these patients have unique AAbs against neuronal antigens often different from the retinal antigens
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