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

Kuo, Yi Chun; Chen, Nancy; Tsai, Rong‐Kung

doi:10.1186/s12886-017-0638-5

Cited by 17 publications

(12 citation statements)

References 27 publications

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“…Matsui et al did demonstrate in AZOOR that at 6 months, the ELM and EZ can regenerate; however, they did not correlate their findings with treatment [24]. Other articles have shown recovery of the EZ and VA after intravitreal injection of dexamethasone (AZOOR) and recovery of the EZ reflectivity with difluprednate (npAIR) [25, 26]. In hydroxychloroquine toxicity, the integrity of the ELM predicts regeneration of the EZ [13].…”

Section: Discussionmentioning

confidence: 99%

Non-paraneoplastic autoimmune retinopathy: multimodal testing characteristics of 13 cases

Khanna

Martins

Oakey

et al. 2019

J Ophthal Inflamm Infect

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Background Non-paraneoplastic autoimmune retinopathy (npAIR) is a rare autoimmune disease that primarily affects retinal photoreceptor function and results in profound and often times permanent vision loss. Delay in diagnosis and treatment initiation may contribute to the poor visual prognosis. Methods A retrospective chart review of all patients diagnosed with autoimmune retinopathy at the University of Wisconsin-Madison Eye Clinics between January 2012 and January 2017 was performed. Twenty eyes of 15 patients had evidence of any form of autoimmune retinopathy through a combination of symptoms, ocular findings, visual fields, optical coherence tomography, fundus autofluorescence, full-field and multifocal electroretinography, and serum anti-retinal antibodies. Clinical records were also analyzed for demographic data, systemic comorbidities, visual acuity, treatment employed, and disease progression. Results We identified 18 eyes from 13 patients who fit the criteria for non-paraneoplastic autoimmune retinopathy. Sixty-nine percent of patients were female with a mean age of symptom onset of 56.9 ± 20.3 years. Sixty-seven percent of eyes had an associated autoimmune condition, most commonly hypothyroidism. Serum testing revealed a preponderance of antibodies against carbonic anhydrase II, while imaging revealed characteristic changes. Fundus autofluorescence most commonly showed hyperautofluorescence around the macula. The delayed diagnosis led to a larger reduction in the horizontal extent of ellipsoid zone in 1-mm perifoveal area on optical coherence tomography with resulting visual decline. There was no difference in the change of visual acuity when stratifying for patients with autoimmune conditions ( p = 0.52) or treatment status ( p = 0.50). None of the patients who received treatment developed contralateral eye involvement or experienced disease progression based on visual acuity or symptoms. Conclusion Non-paraneoplastic autoimmune retinopathy has a wide and often challenging to diagnose spectrum of clinical symptoms and imaging findings. Immunosuppressive therapy can be considered empiric in the face of a suggestive presentation and can be initiated after an evaluation of clinical findings and multimodal testing, though treatment does not appear to affect regeneration of the ellipsoid zone on OCT or impact visual acuity. Treatment should be primarily used to prevent disease progression and contralateral eye involvement. Trial registration N/A

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Section: Discussionmentioning

confidence: 99%

Non-paraneoplastic autoimmune retinopathy: multimodal testing characteristics of 13 cases

Khanna

Martins

Oakey

et al. 2019

J Ophthal Inflamm Infect

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“…[ 2 ] Although an association between AZOOR and central nervous system (CNS) demyelinating lesions has been suggested,[ 3 ] misdiagnosis of AZOOR as optic neuritis is not uncommon. [ 4 ] A few cases of AZOOR have been previously reported in Taiwan;[ 5 6 ] here, we present a case of AZOOR presenting clinical features mimicking optic neuritis.…”

Section: Introductionmentioning

confidence: 80%

“…[ 2 ] Steroids have been reported to be effective for treating AZOOR. [ 5 6 17 ] Chen et al . reported satisfactory visual outcomes after systemic steroid treatment in nine patients with AZOOR.…”

Section: Discussionmentioning

confidence: 99%

Acute zonal occult outer retinopathy presenting as optic neuritis

Peng¹,

Lee²,

Cheng

et al. 2020

Taiwan J Ophthalmol

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Acute zonal occult outer retinopathy (AZOOR) is an outer retinal disorder characterized by the acute loss of visual functions. Herein, we report a case of AZOOR presenting features mimicking optic neuritis. A 17-year-old healthy male reported fogginess in the right eye for 2 weeks. His best-corrected visual acuity was 20/20 in both eyes. Results of a color vision test and pupillary reaction were unremarkable. Funduscopic examination revealed a subtle hyperemic disc surrounded by hyperpigmentation in the right eye. Visual field examination confirmed an enlarged blind spot in the affected eye. Fundus autofluorescence imaging revealed zonal hyperautofluorescence around the optic disc. Fluorescein angiography showed optic disc staining and a window defect in the retinal pigment epithelium. Optical coherence tomography demonstrated loss of the ellipsoid line at the corresponding hyperautofluorescent region. All these characteristics indicated a diagnosis of AZOOR. However, the prolonged P100 wave observed through visual-evoked potential examination, hyperintensity T2 signal at the retrobulbar optic nerve through magnetic resonance imaging, and mild hyperemic optic disc along with optic disc staining through fluorescein angiography resemble the characteristics of optic neuritis. Because the clinical features of AZOOR are similar to those of optic neuritis, ophthalmologists should be able to differentiate between these two diseases to achieve a timely and correct diagnosis.

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“…5,6 Systemic corticosteroids are subsequently used as mainstay therapy, while the use of combinations of non-corticosteroid systemic immunosuppressive drugs with steroids has been reported in isolated cases, with variable results. [7][8][9] Thus, the management of these patients is still a challenge, since no treatment has proved effective either in reducing symptoms or in recovering visual field loss.…”

Section: Discussionmentioning

confidence: 99%

Long term follow-up and effect of immunosuppression in acute zonal occult outer retinopathy

Guijarro

Muñoz

Alejandre

et al. 2020

European Journal of Ophthalmology

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Acute zonal occult outer retinopathy (AZOOR) is a rare syndrome characterized by sudden onset of photopsia, scotomas, and abnormal electrophysiological tests, predominantly affecting young women. Although its pathogenesis remains unknown, auto-reactivity to retinal components is thought to mediate tissue damage. A 42-year-old woman presented with symptoms and examination consistent with the diagnosis of AZOOR. She was treated with azathioprine for 5 years. In spite of the immunosuppressive treatment, clear progression in the visual field, autofluorescence, electrophysiological tests and optical coherence tomography was observed. Treatment with intravenous immunoglobulins (IVIg) and subcutaneous Abatacept was subsequently started with little efficacy. Hereby, we present a case of progressive AZOOR despite aggressive immunosuppression with 10-year follow up. Currently, there is no consensus regarding management of AZOOR, and the convenience of administering aggressive immunosuppression remains uncertain.

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

Cited by 17 publications

References 27 publications

Non-paraneoplastic autoimmune retinopathy: multimodal testing characteristics of 13 cases

Non-paraneoplastic autoimmune retinopathy: multimodal testing characteristics of 13 cases

Acute zonal occult outer retinopathy presenting as optic neuritis

Long term follow-up and effect of immunosuppression in acute zonal occult outer retinopathy

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