However, it is probable that AIR is more prevalent than thought and remains undiagnosed in many cases due to the lack of standardized diagnostic criteria and its protean clinical features that overlap with other retinal degenerative diseases. Although the prevalence of AIR is unknown, it is thought to be a rare entity. The diagnosis of AIR is typically made based on the presence of antiretinal antibodies and a combination of certain clinical features, in the absence of another cause of symptoms. As autoimmune retinopathy (AIR) is the preferred term for an acquired and presumed immune-mediated retinopathy due to antiretinal autoantibodies in the absence of a malignancy, we use AIR to indicate the nonparaneoplastic form of autoimmune retinopathy unless otherwise indicated.ĭespite being described almost 20 years ago, 1 AIR remains an ill-defined disease. Autoimmune retinopathies can be categorized as paraneoplastic AIR (pAIR), which includes cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR), or nonparaneoplastic autoimmune retinopathy in the absence of malignancy. Consensus agreed that an ideal assay should have a two-tier design and that western blot (WB) and immunohistochemistry (IHC) should be the methods used to identify antiretinal antibodies.Īutoimmune retinopathies are a group of inflammatory-mediated diseases characterized by the presence of antiretinal antibodies, visual field deficits, and photoreceptor dysfunction in the setting of progressive otherwise unexplained vision loss. Regarding antiretinal antibody detection, consensus agreed that a standardized assay system is needed to detect serum antiretinal antibodies.
Experts agreed that more evidence is needed to treat nonparaneoplastic AIR patients with long-term immunomodulatory therapy and that there is enough equipoise to justify randomized, placebo-controlled trials to determine if nonparaneoplastic AIR patients should be treated with long-term immunomodulatory therapy.
Diagnostic criteria and tests essential to the diagnosis of nonparaneoplastic AIR and multiple supportive criteria reached consensus.įor treatment, experts agreed that corticosteroids and conventional immunosuppressives should be used (prescribed) as 1 st or 2 nd line treatments, though a consensus agreed that biologics and intravenous immunoglobulin were considered appropriate in the treatment of nonparaneoplastic AIR patients regardless of the stage of disease. There was unanimous agreement among experts regarding the categorization of autoimmune retinopathies as nonparaneoplastic and paraneoplastic, including cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR).
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