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Background: The purpose of this study is to report two new cases of Usher syndrome associated with Fuchs' heterochromic uveitis (FHU), to confirm our previous observation of the association between FHU and retinitis pigmentosa (RP), and to evaluate if FHU is particularly associated with Usher syndrome., Methods: Retrospective medical record review of all new RP cases at Hadassah Medical Center between the years 2000 and 2007, review of our previously published data, and a meta-analysis of published relevant articles in peer reviewed journals., Results: During the time frame of the study we diagnosed 58 new cases of RP, of whom one male and one female had the typical findings of FHU, and both had Usher syndrome type II. The difference in the occurrence of FHU between the 616 controls and the patients with RP was significant (p = 0.0073, Fisher's exact test). In our combined data, FHU occurred only in two types of RP; RP simplex with an incidence of 0.57%, and Usher syndrome with an incidence of 13.5%. This difference between the incidence of FHU in patients with Usher syndrome and other types of RP was significant (p < 0.0001, Fisher's exact test). Adding up these two cases with what is already published in the literature makes up a total of 17 RP patients with coexisting FHU., Conclusions: This study confirms the association between FHU and RP; and a particularly stronger association with Usher syndrome type II. Although infectious agents seem to play a role, the cause for this significant correlation is still unclear.

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: To explore the effect of pregnancy on the clinical course, outcome, and treatment in multiparous women with non-infectious uveitis. Methods: Retrospective study of women with a history of non-infectious uveitis and pregnancies prior to and during disease course. Disease activity and severity 1-year prior pregnancy, during pregnancy, and 1-year postpartum were recorded as well as patients' and diseases' characteristics. The main outcome measures included the rate and severity of uveitis attacks and the effect on ocular complications and therapies. Results: Included were 32 women (70 pregnancies, mean of 2.6 pregnancies/patient), with a mean follow-up time of 6.5 years. The most common uveitis types were anterior (31%) and pan-uveitis (31%). Flare-ups were more frequent in the year prior to pregnancy, in the first trimester, and in the postpartum period and decreased markedly during pregnancy. Women who experienced a flare-up during pregnancy had a higher rate of flare-ups in the year prior pregnancy than those who did not experience a flare-up during pregnancy (p-0.047). The rate of flare-ups 12 months' postpartum was also higher compared to women without any flare-up during pregnancy (p = 0.01). Severity of flare-ups in the postpartum period was worse in women who experienced a flare-up during pregnancy compared to women without flare-ups (p = 0.001). The severity of flare-ups was higher in the first pregnancy compared to subsequent pregnancies. Conclusions: Women who had active or non-controlled uveitis prior to pregnancy have higher disease activity and severity during pregnancy as well. The first pregnancy seems to behave differently from subsequent pregnancies, in terms of disease severity. [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]

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]

Introduction: The EyeCOPE study characterized noninfectious intermediate posterior, or panuveitis (NIIPPU) before biologic agents were widely available. Methods: This retrospective, observational study included adults with NIIPPU attending a routine ophthalmological visit. Data were collected from the study visit and medical records. Results: Of 565 patients, 58.8% were female, and the mean age was 41.3 years; 33.8% had idiopathic uveitis and 45.8% had panuveitis. The median time from symptom onset to diagnosis and treatment was 27.0 and 30.5 days, respectively. Patients received immunosuppressants and systemic/local corticosteroids. Most patients experienced substantial decline in ocular function (mean best corrected visual acuity, 0.4 logMAR). Mean total work productivity impairment among employed patients was 31.0%. Most patients reported ocular complications (70.8%) such as vision loss and cataracts. Conclusions: Despite treatment, most patients with NIIPPU experienced a decline in ocular function and ocular complications. There is an unmet need for additional NIIPPU treatment, such as targeted monoclonal antibodies. [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]

Ocular inflammation resulting from a lens pathology is rare in the absence of a cataract or lens trauma because of the lens' immune privilege. The lens can be a source of ocular inflammation when the capsule is broken or when lens proteins leak out through an intact capsule. These uveitides are termed lens-induced uveitis (LIU) and are often associated with advanced cataracts. Cataracts are part of the normal aging process, and in today's world, cataract surgery is a safe and affordable means of vision restoration in the developed world. In patients with neglected cataracts and in the developing world where cataract surgery rates are lower, LIU rates are higher together with the associated complications. In this literature review, we intend to equip the armamentarium of the practicing ophthalmologist with an updated knowledge on the demographic features, clinical characteristics, treatment options, and outcomes of LIU. This is to highlight the need for timely management of cataracts before the development of advanced cataracts and LIU. [ABSTRACT FROM AUTHOR]

Purpose: To describe the demographics and clinical characteristics of patients with herpetic anterior uveitis (HAU), and compare characteristics by pathogen, recurrence, and association to iris atrophy.Methods: Multicenter, retrospective study of AU patients diagnosed clinically and by polymerase chain reaction (PCR).Results: The study included 112 eyes in 109 patients: 54 (48.2%) HSV, 34 (30.4%) VZV, 2 (1.8%) CMV, and 22 (19.6%) unspecified diagnosis. HSV eyes, compared to VZV, had a higher recurrence rate, corneal involvement, KPs, iris atrophy, elevated IOP and posterior synechia (p < 0.05). VZV patients had more frequent immunomodulatory treatments and history of systemic herpetic disease (p < 0.05). Fifty-nine (52.7%) eyes had recurrent disease. Iris atrophy was associated with a higher prevalence of posterior synechia, dilated distorted pupil, and high IOP (p < 0.05).Conclusion: Different HAU-causing Herpesviridae produce common clinical findings; therefore, PCR should be used more often to confirm specific diagnosis. Iris atrophy was associated with more severe disease. [ABSTRACT FROM AUTHOR]

Background: The purpose of this study is to report two new cases of Usher syndrome associated with Fuchs’ heterochromic uveitis (FHU), to confirm our previous observation of the association between FHU and retinitis pigmentosa (RP), and to evaluate if FHU is particularly associated with Usher syndrome. Methods: Retrospective medical record review of all new RP cases at Hadassah Medical Center between the years 2000 and 2007, review of our previously published data, and a meta-analysis of published relevant articles in peer reviewed journals. Results: During the time frame of the study we diagnosed 58 new cases of RP, of whom one male and one female had the typical findings of FHU, and both had Usher syndrome type II. The difference in the occurrence of FHU between the 616 controls and the patients with RP was significant ( p = 0.0073, Fisher's exact test). In our combined data, FHU occurred only in two types of RP; RP simplex with an incidence of 0.57%, and Usher syndrome with an incidence of 13.5%. This difference between the incidence of FHU in patients with Usher syndrome and other types of RP was significant ( p < 0.0001, Fisher's exact test). Adding up these two cases with what is already published in the literature makes up a total of 17 RP patients with coexisting FHU. Conclusions: This study confirms the association between FHU and RP; and a particularly stronger association with Usher syndrome type II. Although infectious agents seem to play a role, the cause for this significant correlation is still unclear. [ABSTRACT FROM AUTHOR]

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]