Is Zonal Occult Outer Retinopathy an Autoimmune Disease?
|Ocular Immunology Laboratory, Casey Eye Institute, School of Medicine, Oregon Health & Science University, Portland, OR, USA|
|Corresponding Author :||Grazyna Adamus, Ph.D.
Oregon Health and ScienceUniversity
Casey Eye Institute, 3181 SW Sam Jackson Park Rd.
Portland, OR97239, USA
E-mail: [email protected]
|Received August 10, 2011; Accepted August 25, 2011; Published September 05, 2011|
|Citation: Adamus G (2011) Is Zonal Occult Outer Retinopathy an Autoimmune Disease? J Clinic Experiment Ophthalmol 2:104e. doi:10.4172/2155-9570.1000104e|
|Copyright: © 2011 Adamus G. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.|
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Acute zonal occult retinopathy (AZOOR) is a rare condition of unknown etiology characterized by an acute visual loss that may be related to rapid loss of one or more zones of the outer retinal function [1-3]. AZOOR typically manifests with asymmetrical visual field loss, persistent photopsia, acuity reduction and often with widespread photoreceptor dysfunction on the electroretinogram (ERG) . This condition mostly affects young women and may be linked to female hormones, although such an association has not been reported. The focus of my laboratory is on the autoimmunity of retinopathy, including cancer-associated retinopathy, melanoma-associated retinopathy, autoimmune retinopathy, and occasionally, AZOOR. I am often asked whether AZOOR is an autoimmune disease and whether this condition is associated with the presence of autoantibodies against retinal antigens. If so, do the autoantibodies play a role in pathogenicity of AZOOR? If one suspects an immunological or autoimmune involvement one should look for signs of inflammatory or autoimmune pathology as a result of failure in the mechanism of antigen-specific immunoregulation, typical of such diseases. AZOOR is a relatively new entity and has not been intensively studied. Two recent reviews suggested that AZOOR is a form of AR [4,5]. In 2003, Jampol and Backer proposed a hypothesis that AZOOR may originated from relatively common non-disease specific of "clusters" genes (at specific genetic loci) that predispose individuals to "autoimmune" diseases in a similar way to other inflammatory diseases of unknown etiology . However, there is no strong evidence supporting this hypothesis. A history of AZOOR-associated immune-mediated inflammatory systemic disease was reported in only ~18% of patients . Treatments with systemic corticosteroids and other systemic immunosuppressive agents have not been effective . However, Grass et al.  reported inflammation that typically develop within several weeks following the onset of AZOOR, possible as an effect of an inflammatory response to the dying photoreceptor cells. Occasionally, weeks or months later, narrowing of the retinal vessels, particularly the retinal arteries, perivascular sheathing and reactive changes in the RPE occur, including hypopigmentation and migration of RPE into the overlying retina similar to that occurring in the zones of receptor cell loss in RP.