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Familial Mediterranean fever (FMF) is a rare autoinflammatory disorder. Ocular involvement is rare. The full spectrum and response to treatment is poorly understood. An 18-year-old girl previously diagnosed with FMF presented with sudden loss of vision in the left eye (LE). Best-corrected visual acuity (BCVA) in the LE was finger counting at 1.5 meters. Angiitis with mild "frosting," hemi-central retinal vein occlusion (HCRVO), and acute outer retina rupture (ORR) were observed in the LE. Systemic steroids were initiated immediately. The ORR was sealed 2 weeks later while vision improved to 6/15 (near vision: J2) 5 months later. No recurrences were observed over 5 years of follow-up. We report a rare manifestation of frosted branch angiitis with concomitant HCRVO and ORR in a young patient with FMF. Closure of ORR was attained and vision recovered after treatment with high-dose steroids. [ABSTRACT FROM AUTHOR]

Purpose: This study aims to evaluate the outcomes of adalimumab (ADA) therapy in patients with refractory punctate inner choroidopathy (PIC) and multifocal choroiditis (MFC). Methods: Demographic and clinical data, including LogMAR best-corrected visual acuity (BCVA), were retrospectively collected. Doses of prednisone, immunomodulatory therapies (IMT), and anti-vascular endothelial growth factor (VEGF) injections before and after baseline (ADA initiation) were recorded, as well as the time to clinical remission, time to first flare, and drug-associated adverse events. Results: Seven patients (4 females, ten eyes) were included. The mean follow-up after baseline was 17.8 ± 11.1 months (range 6–33). The mean LogMAR BCVA was 0.35 ± 0.77 at 6 months before baseline and remained stable throughout 12 months after baseline (0.31 ± 0.46 at 12 months; p = 0.47). The mean dose of prednisone decreased from 17.3 ± 19.6 mg/day 6 months before baseline (range 0–60) to 2.6 ± 2.4 mg/day at the last follow-up (range 0–6, p = 0.03). The mean number of flares decreased significantly from 1.43 ± 0.79 over a 6-month period before baseline to 0.2 ± 0.45 (p = 0.02) at 6–12 months after baseline. The mean number of anti-VEGF injections was 4.17 ± 3.92 over the 12-month period before baseline, and it was 2.17 ± 3.06 (p = 0.31) during the first 12 months after baseline. No adalimumab-related adverse events were noted. Conclusion: Adalimumab therapy for refractory PIC/MFC enabled a significant steroid-sparing effect, decreased disease flares, and preserved vision over a mean follow-up of 17.8 months. [ABSTRACT FROM AUTHOR]

We report the visual and clinical outcomes of a middle-aged woman who presented with exudative retinal detachment (ERD) secondary to a vasoproliferative tumor (VPT) in an eye with sarcoidosis-associated intermediate uveitis. A 55-year-old woman previously diagnosed with sarcoidosis presented with decreased vision in the left eye (LE). Visual acuity in the LE was counting fingers. She had active vitritis, and a peripheral retinal vascular mass was noted in the superotemporal periphery. The mass was associated with ERD involving the posterior pole. The patient was managed with systemic and intravitreal steroids, and cyclosporine was subsequently added as a steroid-sparing agent. Because of recurrence of ERD, the patient underwent pars plana vitrectomy, and cryotherapy and laser photocoagulation were applied to the VPT. Two months postoperatively, visual acuity in the LE improved to 6/10. There was marked regression of the VPT and total resolution of the ERD. In conclusion, we report a favorable visual and clinical outcome in a patient with VPT-associated ERD who responded to a combination of medical therapy and surgical intervention. VPT may lead to different remote complications, so timely diagnosis of these tumors and proper management of their complications is warranted. [ABSTRACT FROM AUTHOR]

Purpose To report a case series of six patients suffering from branch retinal artery occlusion due to Bartonella henselae infection, in order to raise awareness to this etiology in the differential diagnosis of retinal artery occlusion. Methods A retrospective case series of patients with branch retinal artery occlusion due to ocular cat scratch disease who presented at four tertiary medical centers in Israel, Turkey and Saudi Arabia between the years 2008-2014. Data retrieved from the medical records included demographic data, exposure, complaints, visual acuity, clinical findings and imaging, laboratory assessment, treatment, disease course and visual outcome. Results The study group consisted of six patients who presented with branch retinal artery occlusion with or without neuroretinitis. One patient had multiple artery occlusions. Diagnosis of cat scratch disease was established based on positive serology and accompanying systemic symptoms, after ruling out other causes of retinal artery occlusion. Treatment included various regimens of antibiotics and systemic steroids. Visual outcome depended upon the obstructed artery. Conclusion Cat scratch disease may cause retinal artery occlusion in infected patients, leaving them with a permanent visual field defect. When retinal artery occlusion occurs as an early sign of the disease, prompt recognition may prevent further events. Thorough history and relevant tests may be of great value. [ABSTRACT FROM AUTHOR]

We describe a case report of pediatric pars planitis complicated with massive exudative retinal detachment (ERD). A 7-year-old presented with visual acuity (VA) in the right eye (RE) of 6/9 and in the left eye (LE) 6/15. Fundoscopy revealed BE inferior retinoschisis, vitritis and snowballs. He was treated with systemic immunosuppressants. RE retinoschisis resolved within 2 months. Three years later presented with LE VA 6/60 and total ERD. Systemic and intravitreal steroids were administered. Due to refractoriness, he underwent 360° scleral buckle and drainage of subretinal fluid. No retinal breaks or traction were detected. Five months postoperatively LE VA was 6/7.5. Long-term stable outcome was maintained. We report a challenging total ERD as a complication of pars planitis. Although extensive and non-responsive to medical therapy, complete resolution and improvement in vision was achieved with surgical intervention and systemic immunosuppression. We speculate that uncontrolled chronic vasculitic process culminated in diffuse ERD. [ABSTRACT FROM AUTHOR]

Background: The use of anti-tumor necrosis factor (TNF)-α agents for uveitis has been increasing since the first efficacy studies in 2004. However, information on their specific role in cystic macular edema remains sparse. The aim of this study was to evaluate the efficacy of anti-TNF-α agents for the treatment of uveitis-related refractory cystoid macular edema. Methods: The files of 23 consecutive patients treated for cystoid macular edema at the uveitis services of two tertiary medical centers in 2006-2011 were reviewed for demographic data, visual acuity, and optical coherence tomography measurements at baseline and 3, 6, and 12 months after treatment. Changes in mean visual acuity and macular thickness were analyzed. Findings were compared between patients treated with a conventional immunosuppressive regimen only ( n = 18, 27 eyes) and patients treated with an anti-TNF-α agent after proving refractory to conventional treatment ( n = 9, 15 eyes). Results: Mean duration of cystoid macular edema before initiation of anti-TNF-α therapy was 12 ± 8 months. The two groups had similar baseline values of mean central macular thickness and visual acuity. Significant improvement in macular thickness was noted at 3 months in both groups, with a maximal effect at 6 months for the anti-TNF-α group ( p = 0.002). Maximal improvement in visual acuity was achieved at 3 months in both groups, with a reduced effect towards 12 months. Mean visual acuity at 12 months was similar in both groups. Conclusions: Anti-TNF-α agents may serve as an effective lasting treatment for long-standing refractory uveitis-related cystoid macular edema. Their role as first-line therapy in this setting warrants further investigation. [ABSTRACT FROM AUTHOR]

We report the long-term follow-up of an immunocompetent patient who presented with slowly progressive endogenous endophthalmitis secondary to Streptococcus anginosus. A 46-year-old healthy man presented with a two-month history of right eye iritis. On examination, visual acuity was 20/60 with intraocular pressure of 6 mm Hg. There was a small layer of hypopyon with non-granulomatous anterior uveitis and vitritis. On funduscopy, fluffy white peripheral retinal and pre-retinal lesions were noted in superonasal periphery. The patient denied any present or past illness. Diagnostic pars plana vitrectomy was performed. Culture and polymerase chain reaction of the vitreous sample were positive for Streptococcus anginosus. Intravitreal vancomycin and ceftazidime and systemic ceftriaxone were administered. Work-up which included blood and urine cultures, chest x-ray, echocardiography and abdominal ultrasound was unyielding. Subsequently and because of persistent post-infectious inflammatory reaction, intravitreal and oral steroids were administered in addition to oral azathioprine later on. After one year of follow-up, visual acuity was 20/20 with near vision of Jaeger 3 + and no signs of active uveitis were seen. Therefore, Streptococcus anginosus should be considered in the differential diagnosis of a slowly progressive endophthalmitis also in immunocompetent individuals. [ABSTRACT FROM AUTHOR]

Purpose: Pars planitis is a commonly observed type of pediatric uveitis. The aim of this study was to evaluate the implications of pars planitis–associated cystoid macular edema (CME) on visual outcome and treatment modalities. Methods: A retrospective review of medical records in a single center with academic practice. Results: Included were 33 children (mean age 8 years, 58 eyes). Eighteen eyes developed CME (31%): in 67% of them, CME was diagnosed at presentation and in 33%, it developed at a mean of 57 months after presentation. Anterior and posterior segment complications were more prevalent in eyes with CME. Papillitis was significantly associated with the development of CME (OR 12.4, 95% CI 2.3 to 65.6, p = 0.003). Patients with CME were 1.7 times more likely to be treated with systemic therapy. By the last follow-up, 50% of patients who never developed CME were without systemic therapy compared with 13% of patients who developed CME (p = 0.034). LogMAR visual acuity improvement between presentation and month 36 was 0.41 for eyes with CME compared with 0.14 for eyes that never developed CME (p = 0.009). Conclusion: Pars planitis–associated CME entailed higher prevalence of ocular complications, more frequent use of immunomodulatory therapy, and a lower rate of remission. [ABSTRACT FROM AUTHOR]

Purpose: To report the epidemiology, etiology, ocular characteristics, treatment and visual outcome of pediatric uveitis in Israel.Methods: Retrospective study from two tertiary uveitis centers.Results: Included were 107 patients (182 eyes), 55% females. Mean age at diagnosis 8.8 years. Uveitis was predominantly anterior, idiopathic, bilateral, and chronic. Systemic associations were seen in 36% of patients of which the most common disease was juvenile idiopathic arthritis. Infectious uveitis accounted for 37% of posterior uveitis cases of which toxoplasmosis was the most common cause. Anterior segment complications were commonly observed at presentation (41%); the most predominant were posterior synechiae, cataract, and band keratopathy. The most common posterior segment complications were papillitis, epiretinal membrane, and macular atrophy/scar. Ninety-three percent of eyes had visual acuity >20/40 at last follow-up.Conclusion: The pattern of pediatric uveitis in Israel is similar to that in the western world. Visual outcome was good in most eyes. [ABSTRACT FROM AUTHOR]

Purpose: To study the long-term clinical outcomes of uveitic glaucoma and to identify risk factors for progression.Methods: Retrospective study of uveitic glaucoma patients in two tertiary medical centers in 2003-2015. Patient- and disease-related data was retrieved. Clinical parameters and visual fields measured at predetermined time points were recorded. Outcome measures included maintaining intraocular pressure ≤21 mmHg and preserving visual fields.Results: Included were 34 patients (53 eyes), with a mean follow-up of 7 years. Idiopathic anterior uveitis and open-angle glaucoma were most common. In total, 62% of eyes were steroid responders. Higher IOP was associated with posterior synechiae, peripheral-anterior synechiae, steroidal, and immunomodulatory therapy (p<0.05). Glaucomatous field defects developed in 49%, with most showing no progression, despite elevation of cup-to-disc ratio (p<0.05).Conclusions: Chronic severe uveitis, expressed by structural complications and immunomodulatory therapy, was associated with high IOP and the need for more IOP lowering medications, but was unrelated to glaucomatous damage. [ABSTRACT FROM AUTHOR]

Purpose: To compare tacrolimus monotherapy with tacrolimus and prednisone therapy for the maintenance of disease remission in subjects with noninfectious posterior segment intraocular inflammation (PSII). Design: Randomized, controlled, phase 2b, open-label, dual-center noninferiority trial. Participants: Fifty-eight patients with sight-threatening PSII. Methods: Patients requiring a second-line systemic immunosuppressive agent to control their PSII were treated with therapeutic doses of oral tacrolimus. Those subjects who subsequently were able to taper their prednisone dose to 10 mg daily without disease reactivation were assigned randomly either to stop prednisone or to continue 7.5 to 10 mg prednisone daily for 9 months. Main Outcome Measures: Change in logarithm of the minimum angle of resolution (logMAR) visual acuity (VA) and rate of patient withdrawal resulting from treatment inefficacy or intolerance. Results: Thirty-five patients successfully tapered their prednisone to 10 mg daily. Of these, 16 were allocated randomly to receive tacrolimus monotherapy and 19 to continue taking prednisone and tacrolimus dual therapy. The difference in the mean change in VA for monotherapy compared with the dual therapy group was less than 1 logMAR letter (logMAR, −0.008; 95% confidence interval, −0.108 to 0.092; P = 0.870). The proportion of patients who tolerated treatment and maintained disease remission for 9 months after randomization also was similar in both groups (monotherapy, 62.5%; dual therapy, 68.4%; P = 0.694). All monotherapy treatment failures were the result of disease reactivation, whereas 50% of dual-therapy failures were the result of drug intolerance. Conclusions: This study provides preliminary evidence that corticosteroids can be withdrawn in tacrolimus-treated patients who are able to achieve control of PSII with 10 mg prednisone daily, and any advantage of dual therapy in the prevention of disease reactivation was offset by its greater treatment intolerance. These findings support the further evaluation of corticosteroid-free treatment in future phase 3 trials (International Standard Randomised Controlled Trial Number Register identification, ISRCTN46576063). Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references. [Copyright &y& Elsevier]

Endophthalmitis after a penetrating trauma occurs in 3% to 30% of cases. Prompt recognition and treatment are paramount to avoid irreversible visual loss. We present a case of severe panuveitis following ocular trauma with a tree branch that did not cause any evident ocular wound and discuss the difficulties in achieving a diagnosis that can allow proper treatment. A healthy 21-year-old man presented with acute anterior uveitis. He was managed elsewhere with oral acyclovir and topical steroids for presumed herpetic uveitis. He subsequently developed severe panuveitis with profound decrease in vision. Diagnostic vitrectomy was performed and vitreous samples were positive for Staphylococcus epidermidis. Systemic and intravitreal antibiotic therapy was initiated and after 5 days, the patient recovered with a remarkable improvement in visual acuity to 6/12. Post-traumatic endophthalmitis can result from an imperceptible trauma with no obvious compromise of the globe. [ABSTRACT FROM AUTHOR]