Retinal Dysfunction Caused by Autoimmune Mechanisms

Autoimmune retinal disorders have been identified, including acute zonal occult outer retinopathy (AZOOR), AZOOR complex, autoimmune retinopathy (AIR) comprising paraneoplastic AIR (pAIR), cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and non-paraneoplastic AIR (npAIR). Patients with autoimmune retinal disorders typically present with sudden or acute onset of photopsia, photophobia, night blindness, rapid visual loss, and visual field abnormalities. The combination of multimodal imaging and electrophysiology is crucial because these diseases are challenging to diagnose. In particular, electroretinograms (ERGs) are essential for diagnosis. However, no treatment has been established to date. Additionally, a case of inner retinal dysfunction, thought to be a type of AIR, was recently reported. The diagnosis is difficult because most cases occur in one eye, and although the patient complains of severe photophobia, retinal imaging is almost normal, vision is preserved and there is almost no progression. The ERG is very characteristic, with cone-rod dysfunction and negative ERG. This chapter describes in detail the characteristics of AZOOR, AIR, and acute inner retinal dysfunction as new phenotypes of AIR