Consensus-based recommendations for optical coherence tomography angiography reporting in uveitis.

BACKGROUND/AIMS: To establish a consensus in the nomenclature for reporting optical coherence tomography angiography (OCTA findings in uveitis. METHODS: The modified Delphi process consisted of two rounds of electronic questionnaires, followed by a face-to-face meeting conducted virtually. Twenty-one items were included for discussion. The three main areas of discussion were: wide field OCTA (WF-OCTA), nomenclature of OCTA findings and OCTA signal attenuation assessment and measurement. Seventeen specialists in uveitis and retinal imaging were selected by the executive committee to constitute the OCTA nomenclature in Uveitis Delphi Study Group. The study endpoint was defined by the degree of consensus for each question: 'strong consensus' was defined as >90% agreement, 'consensus' as 85%-90% and 'near consensus' as >80% but <85%. RESULTS: There was a strong consensus to apply the term 'wide field' to OCTA images measuring over 70° of field of view, to use the terms 'flow deficit' and 'non-detectable flow signal' to describe abnormal OCTA flow signal secondary to slow flow and to vessels displacement respectively, to use the terms 'loose' and 'dense' to describe the appearance of inflammatory choroidal neovascularisation, and to use the percentage of flow signal decrease to measure OCTA ischaemia with a threshold greater than or equal to 30% as a 'large area'. CONCLUSIONS: This study sets up consensus recommendations for reporting OCTA findings in uveitis by an expert panel, which may prove suitable for use in routine clinical care and clinical trials.

Optical coherence tomography diagnostic signs in posterior uveitis.

A diagnostic sign refers to a quantifiable biological parameter that is measured and evaluated as an indicator of normal biological, pathogenic, or pharmacologic responses to a therapeutic intervention. When used in translational research discussions, the term itself often alludes to a sign used to accelerate or aid in diagnosis or monitoring and provide insight into "personalized" medicine. Many new diagnostic signs are being developed that involve imaging technology. Optical coherence tomography is an imaging technique that provides in vivo quasi-histological images of the ocular tissues and as such it's able to capture the structural and functional modifications that accompany inflammation and infection of the posterior part of the eye. From the hyperreflective inflammatory cells and deposits in the vitreous and on the hyaloid, to the swollen photoreceptors bodies in multiple evanescent white dots syndrome, and from optical differences in the subretinal fluid compartments in Vogt-Koyanagi-Harada disease to the hyporeflective granulomas in the choroid, these tomographical signs can be validated to reach the status of biomarkers. Such non-invasive imaging diagnostic signs of inflammation can be very useful to clinicians seeking to make a diagnosis and can represent a dataset for machine learning to offer a more empirical approach to the detection of posterior uveitis.

ACCELERATED ONSET OF RETINAL TOXICITY FROM HYDROXYCHLOROQUINE USE WITH CONCOMITANT BREAST CANCER THERAPY.

PURPOSE: To report a case of accelerated retinal toxicity due to hydroxychloroquine (HCQ) use for treatment of Sjögren syndrome in a patient treated with concomitant chemotherapy for breast cancer. METHODS: Observational case report. RESULTS: A 56-year-old white woman using 400 mg HCQ (7.1 mg/kg real body weight) daily for a total of 2 years and 10 months for treatment of Sjögren syndrome with concomitant use of docetaxel and cyclophosphamide therapy (21-day cycle, 4 cycles) followed by anastrozole for breast cancer, presented with visual complaints and findings of severe HCQ toxicity. CONCLUSION: Concomitant breast cancer therapy may have a synergistic effect with HCQ leading to accelerated retinal toxicity. As such potential acceleration is poorly understood, patients on HCQ who are treated with concomitant chemotherapy should be considered for more frequent retinal screenings to maximize safety and preservation of vision.

Remember the retina: retinal disorders presenting to neurologists.

General neurologists and stroke specialists are regularly referred cases of visual disturbance by general practitioners, emergency doctors and even ophthalmologists. Particularly when the referral comes from ophthalmologists, our assessment tends to focus on the optic nerve; however, retinal conditions may mimic optic neuropathy and are easily missed. Their diagnosis requires specific investigations that are rarely available in a neurology clinic. This article focuses on how a general neurologist can identify retinal problems from the clinical assessment and how to proceed with initial investigations. The following cases were all referred to a consultant neurologist (GTP) from ophthalmology services as optic neuropathies or other neurological disorders. Part A of the summary describes the presentation and findings in the neurology clinic; part B describes the subsequent specialist assessment in the neuro-ophthalmology/eye clinic.

Gevokizumab in the Treatment of Autoimmune Non-necrotizing Anterior Scleritis: Results of a Phase I/II Clinical Trial.

PURPOSE: To evaluate the safety and potential efficacy of gevokizumab, an anti-interleukin 1ß (IL-1ß) monoclonal antibody, in the treatment of active, noninfectious, non-necrotizing anterior scleritis. DESIGN: Phase 1/2, open label, nonrandomized, prospective, single-arm pilot trial. METHODS: Eight patients with active, noninfectious, non-necrotizing anterior scleritis with a scleral inflammatory grade of +1 to +3 in at least 1 eye were enrolled. In 1 patient both eyes were enrolled, for a total of 9 eyes (4 eyes with +1, 1 eye with +2, and 4 eyes with +3). Patients received 1 subcutaneous injection of 60 mg gevokizumab at baseline and then every 4 weeks for 12 weeks. Complete physical and ocular examinations were performed at each visit. The primary outcome was at least a 2-step reduction or reduction to grade 0 in scleral inflammation on a 0 to +4 scale according to a standardized photographic scleritis grading system by 16 weeks in the study eye compared to baseline. Secondary outcomes included changes in visual acuity, intraocular pressure, and trends in scleral grading. Participants who met the primary outcome were eligible to continue in the study for up to 52 weeks and received additional gevokizumab injections every 4 weeks until week 36, followed by 2 safety visits at weeks 40 and 52. RESULTS: Seven eyes from 7 patients met the primary outcome within a median time of 2 weeks following the first gevokizumab injection. No definitive changes in visual acuity or intraocular pressure were identified. There were no serious adverse events related to the study drug. A total of 43 adverse effects were reported, with 93% described as mild, 95% as nonocular, and only 14% deemed possibly caused by the investigational treatment. CONCLUSIONS: The results of this small study suggest that blockage of IL-1ß using gevokizumab may be beneficial in treating active, noninfectious anterior scleritis and that gevokizumab is well tolerated. Larger randomized trials are warranted to assess the true efficacy of gevokizumab in the treatment of non-necrotizing anterior scleritis.

The Association of Statin Use with Age-Related Macular Degeneration Progression: The Age-Related Eye Disease Study 2 Report Number 9.

PURPOSE: To evaluate the association of statin use with progression of age-related macular degeneration (AMD). DESIGN: Preplanned, prospective cohort study within a controlled clinical trial of oral supplementation for age-related eye diseases. PARTICIPANTS: Age-Related Eye Disease Study 2 (AREDS2) participants, aged 50 to 85 years. METHODS: Factors, including age, gender, smoking status, aspirin use, and history of diabetes, hypertension, heart disease, angina, and stroke-all known to be associated with statin use-were included in a logistic regression model to estimate propensity scores for each participant. Age-adjusted proportional hazards regression models, with and without propensity score matching, were performed to evaluate the association of statin use with progression to late AMD. Analyses adjusting for the competing risk of death were also performed. MAIN OUTCOME MEASURES: Baseline and annual stereoscopic fundus photographs were assessed centrally by masked graders for the development of late AMD, either neovascular AMD or geographic atrophy (GA). RESULTS: Of the 3791 participants (2462 with bilateral large drusen and 1329 with unilateral late AMD at baseline), 1659 (43.8%) were statin users. The overall analysis, with no matching of propensity scores and no adjustment for death as a competing risk, showed that statin use was not associated with progression to late AMD (hazard ratio [HR], 1.08; 95% confidence interval [CI], 0.83-1.41; P = 0.56). When matched for propensity scores and adjusted for death as a competing risk, the result was not statistically significant (HR, 0.81; 95% CI, 0.55-1.20; P = 0.29). Furthermore, subgroup analyses of persons with or without late AMD at baseline and the various components of late AMD (neovascular AMD, central GA, or any GA) also showed no statistically significant association of statin use with progression to AMD. CONCLUSIONS: Statin use was not statistically significantly associated with progression to late AMD in the AREDS2 participants, and these findings are consistent with findings in the majority of previous studies. Statins have been demonstrated to reduce the risk of cardiovascular disease, but our data do not provide evidence of a beneficial effect on slowing AMD progression.

Idiopathic multifocal choroiditis/punctate inner choroidopathy with acute photoreceptor loss or dysfunction out of proportion to clinically visible lesions.

PURPOSE: To report acute/subacute vision loss and paracentral scotomata in patients with idiopathic multifocal choroiditis/punctate inner choroidopathy due to large zones of acute photoreceptor attenuation surrounding the chorioretinal lesions. METHODS: Multimodal imaging case series. RESULTS: Six women and 2 men were included (mean age, 31.5 ± 5.8 years). Vision ranged from 20/20-1 to hand motion (mean, 20/364). Spectral domain optical coherence tomography demonstrated extensive attenuation of the external limiting membrane, ellipsoid and interdigitation zones, adjacent to the visible multifocal choroiditis/punctate inner choroidopathy lesions. The corresponding areas were hyperautofluorescent on fundus autofluorescence and were associated with corresponding visual field defects. Full-field electroretinogram (available in three cases) showed markedly decreased cone/rod response, and multifocal electroretinogram revealed reduced amplitudes and increased implicit times in two cases. Three patients received no treatment, the remaining were treated with oral corticosteroids (n = 4), oral acyclovir/valacyclovir (n = 2), intravitreal/posterior subtenon triamcinolone acetate (n = 3), and anti-vascular endothelial growth factor (n = 2). Visual recovery occurred in only three cases of whom two were treated. Varying morphological recovery was found in six cases, associated with decrease in hyperautofluorescence on fundus autofluorescence. CONCLUSION: Multifocal choroiditis/punctate inner choroidopathy can present with transient or permanent central photoreceptor attenuation/loss. This presentation is likely a variant of multifocal choroiditis/punctate inner choroidopathy with chorioretinal atrophy. Associated changes are best evaluated using multimodal imaging.

Bevacizumab (avastin) for corneal neovascularization--corneal light shield soaked application.

PURPOSE: To describe a case where topical bevacizumab (Avastin) was used in an attempt to reduce corneal neovascularization before corneal graft. METHODS: Topical bevacizumab was applied through a corneal light shield for 20 minutes once a week for 11 weeks to the cornea. RESULTS: Corneal vascularization was not reduced. CONCLUSIONS: Topical bevacizumab was not effective in this case at reducing corneal neovascularization.