Blue-Light Fundus Autofluorescence (BAF), an Essential Modality for the Evaluation of Inflammatory Diseases of the Photoreceptors: An Imaging Narrative.

Our purpose is to describe blue-light fundus autofluorescence (BAF) features of inflammatory diseases of the outer retina characterised by photoreceptor damage. BAF from patients diagnosed with secondary and primary inflammatory photoreceptor damage were retrospectively analyzed and compared to other imaging modalities including fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral domain optical coherence tomography (SD-OCT). Multiple evanescent white dot syndrome (MEWDS), idiopathic multifocal choroiditis (MFC), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and acute syphilitic posterior placoid chorioretinitis (ASPPC), all cases corresponding to secondary photoreceptor diseases caused by inflammatory choriocapillaris nonperfusion, were included and compared to primary photoreceptor disease entities, including acute zonal occult outer retinopathy (AZOOR) and cancer-associated retinopathy (CAR). Both groups showed increased BAFs of variable intensity. In severe cases of APMPPE and ASPPC, BAF also showed hypoautofluorescent areas. In group 1 (secondary diseases) BAF hyperautofluorescent areas were associated with colocalized ICGA hypofluorescent areas, indicating choriocapillaris nonperfusion; whereas in group 2 (primary diseases), no ICGA signs were detected. The associated colocalized areas of hypofluorescence on ICGA in the first group, which were absent in the second group, were crucial to allow the differentiation between primary (photoreceptoritis) and secondary (choriocapillaritis) photoreceptor diseases. BAF patterns in inflammatory diseases of the outer retina can give relevant information on the photoreceptor and RPE involvement, with ICGA being crucial to detect concurring choriocapillaris damage and differentiating the two pathologies.

Acute zonal occult outer retinopathy-like presentation secondary to scleral buckle.

Purpose: To describe a case of acute zonal occult outer retinopathy-like (AZOOR-like) presentation following scleral buckle surgery for rhegmatogenous retinal detachment. Observations: A 48-year-old man underwent successful scleral buckle with cryotherapy for repair of a left eye inferior macula-on rhegmatogenous retinal detachment. Five years later he presented with a six-month history of left peripheral field restriction. Fundus autofluorescence and optical coherence tomography demonstrated degeneration of the photoreceptors in a ring pattern around the left macula. Humphrey visual fields showed functional loss corelating with the imaging, with a paracentral ring scotoma. Electrophysiology demonstrated a delayed 30 Hz flicker latency in the left eye confirming cone system dysfunction. Conclusion and Importance: Scleral buckling surgery for repair of a rhegmatogenous retinal detachment may be associated with a late AZOOR-like presentation.

Atypical Syphilitic Outer Retinitis and Severe Retinal Vasculitis as Onset Manifestations in a Patient with Concurrent HIV and Syphilis Infection.

INTRODUCTION: The most distinctive patterns of posterior pole affectation in syphilitic patients are acute posterior placoid chorioretinitis (ASPPC), pseudoretinitis pigmentosa and panuveitis with white focal preretinal opacities. However, outer retinitis is not a common presenting feature in this disease. CASE REPORT: Thus, we report an atypical case of syphilitic outer retinitis (SOR) and severe retinal phlebitis as presenting manifestations in a patient with HIV and syphilis coinfection. We consider that this patient had mixed characteristics of SOR and ASPPC with features of acute zonal occult outer retinopathy (AZOOR) spectrum, which has only rarely been published in recent years. CONCLUSION: Prompt and appropriate antibiotic treatment permitted total restoration of the external retinal layers, resolution of retinal vasculitis and recuperation of visual acuity. Since SOR is treatable in contrast to AZOOR, ophthalmologists should be aware that SOR needs to be ruled out when making a diagnosis of AZOOR.

Empleo del análisis multimodal para el diagnóstico y seguimiento de un caso de retinopatía aguda zonal oculta externa (AZOOR) / Use of multimodal analysis in the diagnosis and follow-up of a case of Acute Zonal Occult Outer Retinopathy (AZOOR)

Con el objetivo de mostrar la utilidad del análisis multimodal en el diagnóstico y seguimiento de los pacientes con AZOOR, presentamos este caso clínico. Se trata de una paciente de 22 años que acudió a urgencias por fotopsias y escotoma paracentral en ojo izquierdo y que fue diagnosticada de AZOOR mediante la realización de diversas pruebas funcionales y estructurales. La evolución del caso fue hacia un empeoramiento estructural inicial seguido de la resolución casi completa de las lesiones objetivadas en las distintas pruebas realizadas con una mejoría sintomática evidente. El análisis multimodal de los casos de AZOOR permite un diagnóstico bastante preciso de esta enfermedad y su diferenciación de otras de aspecto similar como los síndromes de múltiples puntos blancos o la coroiditis multifocal (AU)

A clinical case is presented in order to show the usefulness of multimodal analysis in the diagnosis and monitoring of patients with Acute Zonal Occult Outer Retinopathy (AZOOR). A 22 year-old patient was seen in the emergency department complaining of photopsia and paracentral scotoma of the left eye. Several structural and functional tests were performed and the patient was diagnosed with AZOOR. The evolution of the case was towards an initial structural worsening, followed by the almost complete resolution of the lesions identified in the different tests carried out, with an obvious symptomatic improvement. Multimodal analysis of AZOOR cases allows a fairly accurate diagnosis of this condition, and its differentiation from others with a similar appearance, such as multiple white point syndromes, or multifocal choroiditis (AU)

Use of multimodal analysis in the diagnosis and follow-up of a case of Acute Zonal Occult Outer Retinopathy (AZOOR).

A clinical case is presented in order to show the usefulness of multimodal analysis in the diagnosis and monitoring of patients with Acute Zonal Occult Outer Retinopathy (AZOOR). A 22 year-old patient was seen in the emergency department complaining of photopsia and paracentral scotoma of the left eye. Several structural and functional tests were performed and the patient was diagnosed with AZOOR. The evolution of the case was towards an initial structural worsening, followed by the almost complete resolution of the lesions identified in the different tests carried out, with an obvious symptomatic improvement. Multimodal analysis of AZOOR cases allows a fairly accurate diagnosis of this condition, and its differentiation from others with a similar appearance, such as multiple white point syndromes, or multifocal choroiditis.

Acute Zonal Occult Outer Retinopathy in a Patient Suffering from Epilepsy: Five-Year Follow-Up.

We report an unprecedented case of a young patient with epilepsy coexisting with acute zonal occult outer retinopathy (AZOOR), a rare white dot syndrome of unknown etiology, associated with damage to the large zones of the outer retina. Recently, it has been established that epileptic episodes contribute to an inflammatory response both in the brain and the retina. A 13-year-old male patient with epilepsy was referred by a neurologist for an ophthalmologic consultation due to a sudden deterioration of visual acuity in the left eye. The examination, with a key role of multimodal imaging including color fundus photography, fluorescein angiography, indocyanine green angiography (ICGA), fundus autofluorescence (FAF), swept-source optical coherence tomography (SS-OCT) with visual field assessment, and electroretinography indicated AZOOR as the underlying entity. Findings at the first admission included enlargement of the blind spot in visual field examination along a typical trizonal pattern, which was revealed by FAF, ICGA, and SS-OCT in the left eye. The right eye exhibited no abnormalities. Seminal follow-up revealed no changes in best corrected visual acuity, and multimodal imaging findings remain unaltered. Thus, no medical intervention is required. Our case and recent laboratory findings suggest a causative link between epilepsy and retinal disorders, although this issue requires further research.

Two case reports of acute zonal occult outer retinopathy (AZOOR): Importance of multimodal diagnosis.

A presentation is made of two cases of acute zonal occult outer retinopathy (AZOOR); one a young man of 19 years, and the other a 42-year-old woman. The young man complained of unilateral scotoma and photopsia. The woman presented with bilateral visual loss and photopsia. Multimodal imaging, including fundus photography, fluorescein angiography, fundus autofluorescence, spectral-domain optical coherence tomography, and visual field testing, supported the diagnosis of AZOOR. The differential diagnosis is complicated, since it has clinical features in common with other retinopathies. This means that it is essential to use modern imaging tests, especially those where the characteristic trizonal pattern is shown, such as in autofluorescence and OCT.

Dos casos de retinopatía externa oculta zonal aguda: importancia del diagnóstico multimodal / Two case reports of acute zonal occult outer retinopathy (AZOOR): importance of multimodal diagnosis

Presentamos dos casos de retinopatía externa oculta zonal aguda (AZOOR). Un varón joven de 19 años de edad y una mujer de 47 años. El varón joven presentó inicialmente clínica unilateral de escotoma centrocecal y fotopsias. La sintomatología inicial de la mujer fue disminución de visión y fotopsias bilaterales. Los estudios realizados mediante imagen multimodal con fotografías del fondo de ojo, angiografía fluoresceínica, autofluorescencia de fondo, tomografía de coherencia óptica de dominio espectral y pruebas de campo visual fueron consistentes con el diagnóstico de AZOOR. El diagnóstico diferencial de esta patología es complicado, ya que comparte datos clínicos con otras retinopatías, por lo que resulta imprescindible apoyarse en las pruebas de imagen disponibles hoy en día, prestando especial atención a aquellas donde puede verse el patrón trizonal característico, como son la autofluorescencia y la OCT (AU)

A presentation is made of two cases of acute zonal occult outer retinopathy (AZOOR); a 19-year-old man, and the other a 42-year-old woman. The young man complained of unilateral scotoma and photopsia. The woman presented with bilateral visual loss and photopsia. Multimodal imaging, including fundus photography, fluorescein angiography, fundus autofluorescence, spectral-domain optical coherence tomography, and visual field testing, supported the diagnosis of AZOOR. The differential diagnosis is complicated, since it has clinical features in common with other retinopathies. This means that it is essential to use modern imaging tests, especially those where the characteristic trizonal pattern is shown, such as in autofluorescence and OCT (AU)

COVID-19 Recombinant mRNA Vaccines and Serious Ocular Inflammatory Side Effects: Real or Coincidence?

PURPOSE: To report two cases; bilateral arteritic anterior ischemic optic neuropathy (AAION) and bilateral acute zonal occult outer retinopathy (AZOOR) after COVID-19 mRNA vaccination. CASE REPORT: The first patient was a 79-year-old female was presented to us 35 days after a sudden bilateral loss of vision, which occurred two days after receiving the second recombinant mRNA vaccine (Pfizer) injection. Temporal artery biopsy was compatible with AAION. At presentation, the best-corrected visual acuity was 20/1250 and 20/40 in the right and left eyes on the Snellen acuity chart, respectively. There was 3+ afferent pupillary defect in the right eye. The anterior segment and posterior segment exams were normal except for pallor of the optic nerve head in both eyes. Intraocular pressure was normal in both eyes. She was diagnosed with bilateral AAION and Subcutaneous tocilizumab 162 mg weekly was recommended with monitoring her ESR, CRP, and IL-6.The second patient was a 33-year-old healthy female who was referred to us for a progressive nasal field defect in her left eye, and for flashes in both eyes. Her symptoms started 10 days after receiving the second recombinant mRNA vaccine (Moderna) injection. Complete bloodwork performed by a uveitis specialist demonstrated high ESR (25) and CRP (19) levels. As a result, she was diagnosed with unilateral AZOOR in her left eye and was subsequently treated with an intravitreal dexamethasone implant in the same eye. At presentation, vision was20/20 in both eyes. The anterior segment and posterior segment exams were completely normal except for the presence of abnormal white reflex in the temporal macula of her left eye. We diagnosed her with bilateral AZOOR. Since she was nursing, intravitreal dexamethasone implant was recommended for the right eye. CONCLUSION: There may be a correlation between ocular inflammatory diseases with autoimmune mechanism and the mRNA COVID-19 vaccination.

Acute Zonal Occult Outer Retinopathy (AZOOR) Results from a Clinicopathological Mechanism Different from Choriocapillaritis Diseases: A Multimodal Imaging Analysis.

BACKGROUND AND AIM: AZOOR is a rare disease characterized by loss of zones of outer retinal function, first described by J Donald Gass in 1993. Symptoms include acute onset photopsias and subjective visual field losses. The syndrome is characterized by a normal fundus appearance, scotomas and electroretinographic changes pointing towards outer retinal dysfunction. Evolution, response to immunosuppressive treatment and outcome are difficult to predict. The aim of this small case series was to identify the morphological changes and sequence of events in AZOOR thanks to multimodal imaging. METHODS: Charts of AZOOR patients seen in the Centre for Ophthalmic Specialized care (COS, Lausanne, Switzerland) were analyzed by multimodal imaging including fundus photography, fluorescein angiography (FA), indocyanine green angiography (ICGA), blue light fundus autofluorescence (BL-FAF) and spectral domain optical coherence tomography (SD-OCT) in addition to a complete ophthalmological examination including visual field testing and microperimetry, as well as OCT angiography (OCT-A) and ganglion-cell complex analysis when available. Cases and Results: Three AZOOR patients with a mean follow-up of 47 ± 25.5 months were included following the clinical definitions laid down by J Donald Gass. The primary damage was identified at the level of the photoreceptor outer segments with an intact choriocapillaris and retinal pigment epithelium (RPE) layer, these structures being only secondarily involved with progression of the disease. CONCLUSION: Although AZOOR has often been included within white dot syndromes, some of which are now known to be choriocapillaris diseases (choriocapillaritis entities), our findings clearly commend to differentiate AZOOR from entities such as MEWDS (Multiple evanescent white dot syndrome), APMPPE (Acute Posterior Multifocal Placoid Pigment Epitheliopathy), MFC (Multifocal Choroiditis) and others, as the damage to photoreceptors is primary in AZOOR (a retinopathy) and secondary in choriocapillaritis (a choriocapillaropathy).

Use of multimodal analysis in the diagnosis and follow-up of a case of Acute Zonal Occult Outer Retinopathy (AZOOR). / Empleo del análisis multimodal para el diagnóstico y seguimiento de un caso de retinopatía aguda zonal oculta externa (AZOOR).

A clinical case is presented in order to show the usefulness of multimodal analysis in the diagnosis and monitoring of patients with Acute Zonal Occult Outer Retinopathy (AZOOR). A 22 year-old patient was seen in the emergency department complaining of photopsia and paracentral scotoma of the left eye. Several structural and functional tests were performed and the patient was diagnosed with AZOOR. The evolution of the case was towards an initial structural worsening, followed by the almost complete resolution of the lesions identified in the different tests carried out, with an obvious symptomatic improvement. Multimodal analysis of AZOOR cases allows a fairly accurate diagnosis of this condition, and its differentiation from others with a similar appearance, such as multiple white point syndromes, or multifocal choroiditis.

Unilaterally extinguished electroretinograms in an eye with normal visual acuity and visual field.

PURPOSE: This study reports a case of unilateral retinopathy with extinguished full-field ERGs (ffERGs), wherein the visual acuity was 16/16 and the visual field was spared. METHODS: Observational case report. RESULTS: A 39-year-old female had developed nyctalopia in her left eye. Two years later, she visited an ophthalmologist who noted a bilaterally reduced pigmentation of the fundus. Her best-corrected visual acuity was 16/16 in both the eyes. Goldmann perimetry demonstrated that her visual field was bilaterally fully spared. ffERGs measurement was performed in accordance with the ISCEV standard protocol and indicated that her right eye was normal. However, all ERG responses were severely attenuated in her left eye. Multifocal ERG responses were found to be normal in the right eye and extinguished in the left eye except for residual responses that were exclusively located at the center. During the 7 years of the follow-up period, the visual field in the left eye, which was once normal, became shaded, and the development of a ring scotoma was identified. The visual field in the right eye is still full. CONCLUSIONS: The pathogenesis of this patient's condition still remains unknown, while unilateral retinitis pigmentosa, unilateral pigmentary retinopathy, acute zonal occult outer retinopathy, and autoimmune retinopathy can all be considered as possible explanations. The uniqueness of this case study is that the extinguished ERG responses are predictive of the functional alteration in the affected eye, when the initial visual acuity and the visual field were normal.

Diffuse Phlebitis in Patients with Syphilitic Outer Retinopathy.

Purpose: To report two cases of syphilitic outer retinopathy that showed diffuse phlebitis on fluorescein angiography (FA).Methods: Retrospective chart review.Results: The two patients had presentation similar to acute zonal occult outer retinopathy (AZOOR), including hyperautofluorescence on fundus autofluorescence and ellipsoid zone disruption on optical coherence tomography. The main difference from AZOOR was the finding of diffuse phlebitis on FA. Both patients recovered well after antibiotic and steroid treatment.Conclusion: FA is especially important for differentiating syphilitic outer retinopathy from AZOOR. Timely diagnosis and treatment with penicillin and cautious use of steroid usually lead to favorable prognosis.

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

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.

Azoor: Retinopatia externa oculta zonal aguda associada com doença autoimune / Azoor: Acute Zonal Occute Outer retinopathy associated with autoimmune disease

Resumo Retinopatia externa oculta zonal aguda (AZOOR) foi descrita pela primeira vez por Gass em 1993 como uma síndrome com perda rápida de uma ou mais zonas extensas dos segmentos externos da retina. Paciente masculino, 35 anos, portador de doença de Crohn, queixando-se de dor ocular eventual e nictalopia em olho direito desde infância. Em uso regular de azatioprina e mesalazina. melhor acuidade visual 20/20 AO. À fundoscopia, lesões hiperpigmentadas em arcada temporal inferior de olho direito, em treliça, acompanhando vasculatura local. Após exclusão de diagnósticos diferenciais chegou-se ao diagnóstico de Azoor. Azoor é uma síndrome idiopática caracterizada por um quadro agudo início de fotopsia, escotoma ou ambos e é tipicamente associado a uma perda persistente de função visual que envolve uma ou mais zonas da retina externa. Apesar dos sintomas clássicos de fotopsia, o paciente em questão teve uma apresentação clínica atípica. Descrevemos um caso ocorrido em indivíduo do sexo masculino em região periférica apresentando afinamento coroidiano e com doença autoimune associada. Dessa forma, acreditamos que é necessária maior investigação para verificar a etiologia da alteração coroideana e da associação com a doença específica.

Abstract Acute zonal occult external retinopathy (AZOOR) was first described by Gass in 1993 as a syndrome with rapid loss of one or more large areas of the external retinal segments. Male, 35 years, with Crohn's disease complaining of occasional eye pain and right eye nictalopia since childhood. In regular use of azathioprine and mesalazine. better visual acuity 20/20 OU. At fundoscopy, hyperpigmented lesions in the right temporal arcade of the right eye, in trellis, accompanying local vasculature. After exclusion of differential diagnoses, Azoor's diagnosis was reached. Azoor is an idiopathic syndrome characterized by an acute onset of photopsy, scotoma or both and is typically associated with a persistent loss of visual function involving one or more areas of the external retina. Despite classic photopsy symptoms, the patient in question had an atypical clinical presentation. We describe a case of a peripheral male with choroidal thinning and associated autoimmune disease. Thus, we believe that further investigation is necessary to verify the etiology of choroidal alteration and its association with the specific disease.

Retinal Diseases that Can Masquerade as Neurological Causes of Vision Loss.

PURPOSE OF REVIEW: This review aims to discuss retinal diseases that may masquerade as neurological causes of vision loss and highlights modern ophthalmic ancillary testing that can help to establish these diagnoses. RECENT FINDINGS: Retinal diseases with signs and symptoms overlapping with neurological causes of vision loss include central serous chorioretinopathy, retinal ischemia, acute macular neuroretinopathy, Acute zonal occult outer retinopathy (AZOOR) complex diseases, paraneoplastic retinopathy, retinal dystrophy, and toxic retinopathy. Diagnosis is facilitated by electrophysiologic studies and multimodal ophthalmic imaging including optical coherence tomography and fundus autofluorescence imaging. Looking into the future, translation of adaptive optics ophthalmoscopy into clinical practice may facilitate early detection of microscopic retinal abnormalities that characterize these conditions. With conventional methods of physical examination, diagnosis of retinal diseases that may masquerade as neurological causes of vision loss can be challenging. Current advance in multimodal ophthalmic imaging along with electrophysiologic studies enhances the provider's ability to make early diagnosis and monitor progression of these conditions.

Ocular Syphilis mimicking Acute Zonal Occult Outer Retinopathy (AZOOR). / Sífilis ocular imitando una retinopatía oculta externa zonal aguda (AZOOR).

Ocular syphilis can simulate various ophthalmic pathologies with multiple possible ophthalmic findings. Timely treatment of these patients can minimize visual damage, but its diagnosis is often a challenge for the ophthalmologist. We review a case report of a 45-year-old man with a rare and atypical clinical presentation of ocular syphilis, its clinical and imaging characteristics, its diagnosis, treatment and evolution. The patient presented with bilateral painless loss of vision, photopsia and peripheral scotomas with about 1 week evolution. There were no signs of ocular inflammation. The complementary ophthalmic study revealed an acute zonal occult outer retinopathy-like ("AZOOR-like") presentation. Ocular syphilis does not always present with ocular inflammation and its presentation as "AZOOR-like" is very rare. So, we should always maintain a high index of suspicion for this condition, as early diagnosis and treatment are essential for a better prognosis.

Retinal dysfunction of syphilitic outer retinopathy.

PURPOSE: We present our findings in two cases of retinal dysfunction caused by syphilitic outer retinopathy. CASE 1: A 59-year-old man visited our clinic complaining of blurred vision in his left eye. Optical coherence tomography (OCT) demonstrated an absence of the ellipsoid zone (EZ) in the left eye. A round yellowish-white lesion was observed in the posterior pole of the left fundus. Fundus autofluorescence (FAF) showed hyperfluorescent areas in the posterior pole of both fundi although no specific ophthalmoscopic findings were seen in the right eye. The amplitudes of the LA 3.0 1 Hz and LA 3.0 30 Hz ERG responses were reduced with better preservation of the rod responses. Based on a strong positivity to the rapid plasma reagin (RPR) assay and the Treponema pallidum hemagglutination (TPHA) test, he was diagnosed with syphilitic outer retinopathy and treated with systemic antibiotics. The treatment resulted in a restoration of the retinal structures and cone function. CASE 2: A 47-year-old man was referred to our clinic complaining of reduced vision in both eyes. Although the ocular fundus appeared normal, FAF showed a diffuse hyperfluorescent area in the posterior pole and multiple hyperfluorescent spots. Indocyanine green angiography showed multiple confluent areas of hypofluorescence. OCT demonstrated irregular EZs in both eyes. The amplitudes of the LA 3.0 1 Hz and LA 3.0 30 Hz ERG responses were slightly reduced with prolonged implicit times. These findings are comparable to the findings in patients with multiple evanescent white dot syndrome. However, the strong positivity to the RPR and TPHA tests led us to diagnose the patient with outer retinopathy caused by syphilis. Systemic administration of antibiotics resulted in the restoration of the retinal structures and retinal function. CONCLUSIONS: Syphilitic outer retinopathy affected the retinal structures and function that can be restored by antibiotic treatments.

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

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.