Patients With an Acute Zonal Occult Outer Retinopathy–like Illness Rapidly Improve With Valacyclovir Treatment

Mahajan, Vinit B.; Stone, Edwin M.

doi:10.1016/j.ajo.2010.05.024

Cited by 28 publications

(11 citation statements)

References 34 publications

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“…[ 1 ] Autoimmune and inflammatory hypotheses were proposed by Jampol and Becker [ 13 , 14 ]. Other possible mechanisms includes fungal infiltration, [ 15 , 16 ] polycythemia vera, [ 17 ] toxic retinopathy, [ 14 , 18 , 19 ] and anti-retinal antibody [ 20 , 21 ]. Heckenlively and Ferreyra [ 22 ] reviewed retinal disorders evoked by autoantibodies and established a clinical term of autoimmune retinopathy (AIR).…”

Section: Discussionmentioning

confidence: 99%

“…Various treatments have been attempted in patients with AZOOR, including systemic corticosteroids, [ 11 , 12 , 24 ] other systemic immunosuppressive agents, [ 4 , 25 ] and antimicrobial agents, [ 19 ] but their effects were not conclusive yet. Based on the proposed mechanisms of anti-retinal antibody, [ 20 – 22 ] AZOOR could be an autoimmune disease with anti-retinal antibody leakage from the disc margin with spread of immune substance under the retina.…”

Section: Discussionmentioning

confidence: 99%

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Acute Zonal Occult Outer Retinopathy (AZOOR): a case report of vision improvement after intravitreal injection of Ozurdex

2017

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BackgroundAZOOR was first described by Gass in 1993 as a syndrome with rapid loss of one or more extensive zones of the outer retinal segments. It is characterized by photopsia, minimal funduscopic changes, and electroretinographic abnormalities. The efficacy of systemic steroids in treating AZOOR has been previously described and advocated by the concept of autoimmune retinopathy. However, the use of intravitreal of sustained-released steroid had not been mentioned to date.Case presentationA 34-year-old man had sudden onset of central scotoma and photopsia in the left eye. His visual acuity continued deteriorating. The visual field defect demonstrated bilateral enlarged blind spots and altitudinal defects. Fluorescein angiography (FA) showed nonspecific retinal inflammation, and an electroretinogram (ERG) illustrated decreased amplitude of the b wave in both eyes. Optical coherence tomography (OCT) examinations revealed parafoveal loss of the photoreceptor inner/outer segment (IS/OS) junction. Therefore, acute zonal occult outer retinopathy (AZOOR) was diagnosed. Although his vision did not improve under the initial treatment of systemic corticosteroid and calcium channel blocker, remarkable improvement was noticed after the intravitreal injection(IVI) of Ozurdex, consistent with the recovered IS/OS junction disruption.ConclusionsWe herein report a typical case of AZOOR, suggesting that the intravitreal injection of steroid may benefit in certain patients.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Acute Zonal Occult Outer Retinopathy (AZOOR): a case report of vision improvement after intravitreal injection of Ozurdex

2017

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“…3 -5 The pathogenesis of the disease was uncertain, but Gass 6 speculated that a virus, entering the retina through the peripapillary area, was the best explanation for the progressive abnormalities in the fundus. Further considerations included his viral hypothesis, 7 an immune-mediated process or toxic retinopathy, [8][9][10] or fungal infections. 11,12 Numerous therapies, including immunosuppressive agents, anti-inflammatory drugs, and antiviral therapy, used singularly and in combination have shown no proven evidence of benefit.…”

mentioning

confidence: 99%

“…11,12 Numerous therapies, including immunosuppressive agents, anti-inflammatory drugs, and antiviral therapy, used singularly and in combination have shown no proven evidence of benefit. 4,7,8,[12][13][14] Since the original report by Gass, 1 the described spectrum of changes in the fundus in numerous studies [3][4][5][15][16][17][18] has varied from a normal appearance to a severely damaged retina. Nonspecific chorioretinal signs of inflammation and the unknown etiology of the condition have led to difficulties in distinguishing AZOOR from other entities such as optic neuropathies, paraneoplastic diseases, autoimmune retinopathies, toxic degenerations, and infectious diseases, including diffuse unilateral subacute neuroretinitis, lymphoma, and a myriad of hereditary chorioretinal diseases (eg, retinitis pigmentosa).…”

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confidence: 99%

Acute Zonal Occult Outer Retinopathy

et al. 2014

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IMPORTANCE We describe the multimodal imaging in a group of patients showing a distinct clinical entity that best represents acute zonal occult outer retinopathy (AZOOR). OBJECTIVE To propose a classification of AZOOR based on clinical fundus and multimodal imaging. DESIGN, SETTING AND PARTICIPANTS A retrospective review of patients diagnosed as having AZOOR at 2 centers. After reviewing more than 400 cases diagnosed or referred to us as AZOOR or AZOOR complex, we assembled 30 cases that fit our current definition; (48 eyes) with a median age at diagnosis of 47 years (age range, 17-86 years) and a mean follow-up period of 39 months. Twenty patients were female. Eighteen patients had initially been seen with bilateral lesions, mostly asymmetric (4 cases were symmetric). Most patients had no remarkable medical or ocular history. The median visual acuity at the time of presentation was 20/25 (range, 20/20 to 20/400). MAIN OUTCOMES AND MEASURES Multimodal imaging, including fundus photography, fluorescein and indocyanine green angiography, fundus autofluorescence imaging, and corresponding eye-tracked spectral-domain coherence tomography imaging. RESULTS Each patient was initially seen with visual symptoms of photopsia and scotoma, and most had a detectable lesion in the fundus evident clinically or detected on multimodal imaging. The clinical appearance of the AZOOR lesions varied depending on their duration and location, but some features were characteristic, including a demarcating line of the progression at the level of the outer retina and a trizonal pattern of sequential involvement of the outer retina, retinal pigment epithelium, and choroid, as well as frequent zonal progression. Advanced cases of AZOOR demonstrated disruption of the inner and outer retina and severe damage or loss of the retinal pigment epithelium and the choroid. CONCLUSIONS AND RELEVANCE A specific definition of AZOOR based on multimodal imaging is proposed to help physicians distinguish it from other diseases of the posterior fundus, including white spot syndromes and autoimmune, hereditary, paraneoplastic, toxic, and other inflammatory retinopathies.

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“…This notion is supported by case reports of ocular human cytomegalovirus reactivation after local ocular immunosuppression with triamcinolone acetonide in immunocompetent patients 32 , 33 , 34 , 35 , 36 . The longevity of the CD8 T cell response in experimental MCMV retinitis is a striking finding and may provide an explanation for recent reports linking certain forms of posterior autoimmune uveitis to herpetic eye infections 37 , 38 …”

Section: Discussionmentioning

confidence: 67%

Kinetics of ocular and systemic antigen‐specific T‐cell responses elicited during murine cytomegalovirus retinitis

et al. 2011

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Cytomegalovirus (CMV) reactivation in the retina of immunocompromized patients is a cause of significant morbidity as it can lead to blindness. The adaptive immune response is critical in controlling murine CMV (MCMV) infection in MCMV-susceptible mouse strains. CD8 + T cells limit systemic viral replication in the acute phase of infection and are essential to contain latent virus. In this study, we provide the first evaluation of the kinetics of anti-viral T-cell responses after subretinal infection with MCMV. The acute response was characterized by a rapid expansion phase, with infiltration of CD8 + T cells into the infected retina, followed by a contraction phase. MCMV-specific T cells displayed biphasic kinetics with a first peak at day 12 and contraction by day 18 followed by sustained recruitment of these cells into the retina at later time points post-infection. MCMV-specific CD8 + T cells were also observed in the draining cervical lymph nodes and the spleen. Presentation of viral epitopes and activation of CD8 + T cells was widespread and could be detected in the spleen and the draining lymph nodes, but not in the retina or iris. Moreover, after intraocular infection, antigen-specific cytotoxic activity was detectable and exhibited kinetics equivalent to those observed after intraperitoneal infection with the same viral dose. These data provide novel insights of how and where immune responses are initiated when viral antigen is present in the subretinal space.

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Patients With an Acute Zonal Occult Outer Retinopathy–like Illness Rapidly Improve With Valacyclovir Treatment

Cited by 28 publications

References 34 publications

Acute Zonal Occult Outer Retinopathy (AZOOR): a case report of vision improvement after intravitreal injection of Ozurdex

Acute Zonal Occult Outer Retinopathy (AZOOR): a case report of vision improvement after intravitreal injection of Ozurdex

Acute Zonal Occult Outer Retinopathy

Kinetics of ocular and systemic antigen‐specific T‐cell responses elicited during murine cytomegalovirus retinitis

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