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Intermediate uveitis (IU) in an intraocular inflammation that predominantly affects the vitreous. It typically manifests as chronic low-grade inflammation, mainly affecting children and young adults. Uncontrolled chronic intraocular inflammation carries a high risk of visual loss. IU, despite being a low-grade inflammation, is among the sight-threatening conditions owing to its intrinsically-associated vision-robbing ocular complications. It can cause early and late-onset ocular complications affecting the anterior and posterior segments of the eye. 40–60% of the patients who suffer from IU will develop at least one ocular complication during the disease course. Prompt management and long-term monitoring are vital for the early detection of ocular complications and timely intervention. Proactive screening programs are essential in children because of the occult nature of IU in the pediatric age group and the high prevalence of complications at the initial visit. This review provides a comprehensive panorama of anterior segment complications, namely, band keratopathy, corneal endotheliopathy, posterior synechiae, and cataract as well as of posterior segment complications including macular edema, retinoschisis, retinal detachment, vitreous hemorrhage, vasoproliferative tumor, optic neuritis, and papillitis, in addition to glaucoma and hypotony. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionPapillophlebitis is a rare condition, manifesting as CRVO in the young adults. We aim to present our experience in managing patients with papillophlebitis.Retrospective review of patients’ medical files.Included were seven patients with a mean presenting age of 24.86 ± 4.4 years and mean follow-up of 40.4 ± 50.5 months. No pre-existing systemic illness was reported by any patient. One patient was subsequently diagnosed to have Behçet disease and another patient was diagnosed with homozygous mutation to MTHFR C6771. On presentation, fluorescein angiograms showed diffuse vascular and optic disc leakage. Four patients presented with papillophlebitis-associated CME, for which they were treated with systemic steroids and intravitreal anti-VEGF injections. One patient showed full recovery. In 3 patients, due to the protracted course of papillophlebitis and refractory CME, adalimumab was added. All 3 patients eventually showed complete resolution of CME. Two of them eventually developed extensive peripheral capillary non-perfusion that was treated with panretinal photocoagulation. Three patients did not develop CME: In two patients, papillophlebitis resolved after a short course of prednisone while in the third patient, papillophlebitis resolved spontaneously. Mean ± SD presenting log MAR VA was 0.2 ± 0.32 and it was 0.057 ± 0.11 at the last follow-up.To the best of our knowledge, this is the first description that suggests a role for TNF-ɑ blockers in the management of patients with recalcitrant papillophlebitis and non-responsive CME. Further studies are needed in order to thoroughly investigate the molecular background of papillophlebitis and clinical outcomes associated with this class of medications. [ABSTRACT FROM AUTHOR]

We describe the design, synthesis, and activity of a potent thiourea-bridged backbone cyclic peptidomimetic known as Clarstatin, comprising a 5-amino acid sequence (Q/D)1-(R/K)2-X3-X4-A5-(Gln/Asp)1-(Arg/Lys)2-AA3-AA4-Ala5-based on a motif called "shared epitope (SE)", specifically present in specific alleles of the HLA-DRB1 gene. This SE binds to a particular site within the proline reach domain (P-domain) of the cell surface-calreticulin (CS-CRT). CS-CRT is a multifunctional endoplasmic reticulum (ER) calcium-binding protein that is located on the cell surface of T cells and triggers innate immune signaling, leading to the development of inflammatory autoimmune diseases. The development of Clarstatin was based on the parent peptide W-G-D1-K2-S3-G4-A5- derived from the active region of the SE. Following the design based on the cycloscan method, the synthesis of Clarstatin was performed by the Fmoc solid phase peptide synthesis (SPPS) method, purified by HPLC to 96% homogeneity, and its structure was confirmed by LC-MS. Clarstatin reduced calcium levels in Jurkat lymphocyte cultures, ameliorated uveitis in vivo in the experimental autoimmune uveitis (EAU) mice model, and was safe upon acute toxicity evaluation. These findings identify Clarstatin as a promising lead compound for future drug development as a novel class of therapeutic agents in the therapy of uveitis. [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]

To report two cases masquerading as TORCH but eventually diagnosed with Enhanced S-cone Syndrome (ESCS). Descriptive case report Case 1: A ten-month-old boy presented with high hypermetropia, strabismus and bilateral chorioretinal pigmented scars with a history of cat scratch of his mother during pregnancy. He was treated for suspected toxoplasma retinitis. Choroidal neovascular membranes (CNV) were diagnosed bilaterally and treated with intravitreal bevacizumab. Genetic testing showed homozygote mutation in NR2E3 gene. Case 2: A two-year old girl presented with bilateral high hypermetropia and strabismus. Funduscopy revealed extrafoveal chorioretinal lesions and surrounding subretinal fibrosis. An elevated titer of anti-toxocara IgG antibodies was detected and managed accordingly. LE CNV was diagnosed and treated with intravitreal bevacizumab. Genetic testing disclosed homozygote mutation in NR2E3. Ocular manifestations in ESCS can be reminiscent to TORCH. CNV may develop with an incidence of 15%. We report the youngest patient with ESCS-associated CNV. [ABSTRACT FROM AUTHOR]

We aimed to determine medium and long-term effects of TNF-α inhibitors in patients with pediatric uveitis. This was a retrospective review of medical charts. Included were 50 patients (84 eyes). Mean age at diagnosis was 7.22 ± 4.04 years. At baseline (time of initiation of biologic therapy), all patients had active uveitis. Complete control of uveitis was achieved in 84.52% (n = 71) of eyes, after a median of 3 months (IQR 2 months). Mean LogMAR BCVA at baseline was 0.23 ± 0.44; it remained stable at 12 and 24 months. At baseline, 64% of patients were treated with oral corticosteroids, this decreased to 29.5% at 12 months (p = 0.001) and to 21.9% at 24 months (p < 0.001). Mean time to prednisone dose of ≤0.2 mg/kg/day was 8.1 ± 2.02 months after baseline. A total of 40.5% of eyes were treated with topical steroids at baseline and this significantly decreased to 5.8% at 12 months. Multiple linear regression model was calculated to predict moderate and severe visual loss; only presenting visual acuity accounted for a unique variance in the model. In conclusion, TNF-α inhibitors achieved rapid disease control while enabling a remarkable steroid-sparing effect in children suffering from chronic uveitis. Presenting visual acuity was the sole predictor of moderate to severe visual loss. [ABSTRACT FROM AUTHOR]

The uveitides are a collection of over 30 diseases characterised by intraocular inflammation. Previous work demonstrated that the agreement among uveitis experts on diagnosis was modest at best with some pairs of experts having chance alone agreement on selected diseases. The Standardisation of Uveitis Nomenclature (SUN) is a17‐year collaboration among experts in uveitis, ocular image grading, informatics, and machine learning to improve clinical and translational uveitis research. The SUN "Developing Classification Criteria for the Uveitides" project used a rigorous, multi‐phase approach to develop classification criteria for 25 of the most common uveitic diseases. The project's phases were: (1) informatics; (2) case collection; (3) case selection; (4) machine learning; and (5) consensus review and publication. The results were classification criteria with a high degree of accuracy (93.3%–99.3% depending on anatomic class of the uveitis), the goal of which is to form the basis for future uveitis research. [ABSTRACT FROM AUTHOR]

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]

Vitamin D plays an important role in both the innate and adaptive immune systems. We review published data on the relationship between uveitis and vitamin D levels or vitamin D-associated gene polymorphisms. A search of the PubMed and Medline databases was conducted to identify relevant articles concerning vitamin D and uveitis. Sixteen studies were included in this review, and the evidence they present, linking low vitamin D levels with uveitis, is compelling. The uveitic entities shown to be modulated by hypovitaminosis D include, but are not limited to, HLA-B27-associated acute anterior uveitis, Vogt-Koyanagi-Harada (VKH) disease, sarcoidosis-associated uveitis, and juvenile idiopathic arthritis-associated uveitis. Specific polymorphisms of vitamin D family genes were found to correlate with uveitis in ankylosing spondylitis, Behçet's disease, VKH, and HLA B27-positive patients. Further understanding of the role of vitamin D, a known regulator of inflammatory processes, in noninfectious uveitis may advance capabilities in the fields of disease prevention and treatment. [ABSTRACT FROM AUTHOR]

Purpose: To develop an algorithm for the diagnosis of Behçet's disease (BD) uveitis based on ocular findings. Methods: Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion. Results: We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis. Conclusion: This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies. [ABSTRACT FROM AUTHOR]

To report on a patient with probable catastrophicantiphospholipid syndrome (CAPS) with eye, brain and skin involvement. Descriptive presentation of the case and of the relevant clinical photographs. A 17-year-old girl presented with fatigue, loss of appetite, arthralgia, lower limb skin ulcers and livedo reticularis. Workup showed anemia, elevated ESR, CRP, and positive anti-phospholipid antibodies. Right eye funduscopy showed cotton-wool spots along the inferotemporal arcade with arteriolar occlusion, perivascular retinal hemorrhages, and diffuse retinal ischemia in the temporal peripheral retina. Fluorescein angiogram confirmed the widespread retinal ischemia. Brain MRI revealed several white matter lacunar infarcts, minute cortical/subcortical hemorrhages and subarachnoidal insular hemorrhage. The patient was treated with immunomodulatory therapy, Enoxaparin and retinal laser photocoagulation. Within 2 months of treatment, the facial livedo reticularis resolved and the leg ulcers markedly improved. CAPS causes multiple organ thrombosis and is associated with high rate of mortality. [ABSTRACT FROM AUTHOR]

Endogenous endophthalmitis (EE) is an ophthalmological emergency. We report the long-term outcome of bilateral methicillin-resistant Staphylococcus aureus EE in a 23-year-old healthy immunocompetent athlete who presented with EE secondary to pelvic abscess and remained with excellent vision. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionTo provide a comprehensive overview of the diagnostic and therapeutic journey of a pediatric patient with persistent sarcoid-associated panuveitis over a 10-year period, who ultimately developed bilateral macular subretinal fibrosis and visual loss.Retrospective case reportThe patient was diagnosed with sarcoidosis after undergoing a transbronchial biopsy. She was followed up because of granulomatous panuveitis, multifocal choroiditis, and papillitis bilaterally. She maintained a stable condition, and visual acuity was 0.3 RE and 0.5 LE. Immunomodulatory therapy included prednisone, methotrexate, and adalimumab. The patient was lost to follow-up for 20 months because of the COVID-19 pandemic. She was represented with active uveitis and was not responding to TNF-ɑ inhibitors (adalimumab and infliximab). Ultimately, the patient's intraocular inflammation was successfully controlled by using intravitreal steroids (Triamcinolone and Fluocinolone acetonide implant). However, the visual outcome was guarded because of bilateral subretinal fibrosis.10% of patients with sarcoidosis-associated uveitis risk blindness in one eye. The index case progressed to sight-robbing bilateral subretinal fibrosis, a rare complication of ocular sarcoidosis despite a combination of conventional and biologic anti-inflammatory therapies. There is a pressing need to develop new treatment agents for refractory non-infectious uveitis. [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]