Is AZOOR an autoimmune disease?

Abstract

Purpose: Acute zonal occult outer retinopathy (AZOOR) is one of the "white dot syndromes" a clinically heterogeneous group of inflammatory chorioretinopathies. The etiology is not yet clear. Methods: We present a 50 years female patient with a prior history of migraine. She experienced progressive visual loss and visual field defects in the last 3 years. Preceding each episode she experienced blue flickering photopsias. Results: Visual acuity was 0,3 in the right eye and 0,6 in the left eye. Biomicroscopy showed a normal anterior segment, fundus exam revealed pigment epithelial atrophy more pronounced in the worse eye. Electrophysiology showed a marked reduction in the photopic ERG in the more affected eye. MRI demonstrated multiple white matter lesions including a corpus callosum location. Lumbar puncture showed oligoclonal bands. Further tests demonstrated hearing impairment. Therapy was instituted during the three years course of the disease with steroids, immune suppressants and plasmapheresis with visual loss being progressive. New photopsia is currently present. Conclusions: The etiology of AZOOR remains unclear. With our patient being one of the few described in the literature with concomitant multiple sclerosis, the question remains on whether there is an underlying common process of inflammatory autoimmune reactions. Whether treatment is possible, remains to be evaluated.

Association of Acute Zonal Occult Outer Retinopathy With Multiple Sclerosis: A Report of 2 Cases

“…AZOOR is frequently associated with autoimmune diseases. However, there are only a few described cases of patients with AZOOR and CNS demyelinating disease, with only 3 cases in the literature of patients with clinically confirmed MS, one with clinically isolated syndrome and one with a CNS inflammatory disorder not typical of MS (Table 1) (1–5). We add to these reports by detailing the unique history of 2 patients with AZOOR and concomitant MS with different disease progressions.…”