AZOOR Defined (Acute Zonal Occult Outer Retinopathy)

Abstract

AbstractPurpose To identify common diagnostic findings in 25 patients with AZOOR, first described by Gass in 1993 as a syndrome with rapid loss of one or more large zones of outer retinal function. We were interested in better defining recog‐nizable diagnostic features and the natural history.Methods Patients were identified by sudden onset of zonal functional loss (scotomata) on kinetic visual fields, and abnormal electroretinograhy. All patients had full clinical examinations, family histories for autoiommune disease and retinal degeneration, kinetic visual fields, autofluoresence imaging, electroretinography, Western blots for anti‐retinal antibodies (ARAs).Results There were unifying patterns among patients; all but 4 patients were women, over half had family members with other autoimmune disorders, the disease had asymmetric presentation in 35% of patients. The ERG was always abnormal in eyes with the scotomata, and in most the photopic b‐wave amplitudes were markedly decreased in eyes with large scotomata. Enlarged blind spots extending into equatorial regions were a strong and common feature of the scotomata, which could be seen on autofluorescence. Pigment deposits were very uncommon. Western blots were very positive with an average of 6.5 immunoreactive anti‐retinal antibody bands (normal <1.2). Negative waveforms, occurred in 3/25 patients, and 30Hz flicker amplitudes abnormal in 19/25 with only 12/25 with delayed ITs. Patients with recent onset had a better response to immuno‐suppressant therapy.Conclusion AZOOR is another atypical retinopathy that has anti‐retinal antibodies as a common feature. The ERG suggests that pathologic anti‐cone antibodies play a key role, and given the pattern of scotomata, the ARAs access the retina via the optic canal or peripapillary region.