**1. Introduction**

Retinal diseases related to autoimmunity include acute zonal occult outer retinopathy (AZOOR), AZOOR-associated diseases, and autoimmune retinopathy (AIR). These disease conditions are often difficult to diagnose even with the recent retinal imaging tests that have been developed, and it is crucial to combine retinal electrophysiological tests and systemic examinations in treating these diseases. The pathogenesis of these diseases remains unclear and they have no established treatment; however, it has been suggested that often-identified anti-retinal antibodies may play a key role.

Although previous reports have shown that autoimmune-mediated retinopathies often involve the outer retinal layers, current studies have revealed a rare type of retinopathy involving the inner retinal layers. Since it is difficult to diagnose this type of retinopathy using retinal imaging examinations, the use of electroretinograms (ERGs) is important. In this chapter, AZOOR and AIR are reviewed, and this unusual disease type is discussed.
