Standardization Of Uveitis Nomenclature Research Articles

Signs and symptoms of uveitis

SummaryUveitis can be a sight‐threatening disease. In ammation of uveal tract can be divided into: anterior, intermediate, posterior, and panuveitis. Blurred vision, ocular pain, photophobia and oaters are some of the symptoms complained by those who are a ected by uveitis. e onset of uveitis can be either acute or insidious, bilateral rather than unilateral. Posterior uveitis is usually associated with vitritis. Anterior chamber cells and are should be graded according to standardized uveitis nomenclature (SUN) working group. Binocular indirect ophthalmoscopy (BIO) score is used to evaluate the severity of vitritis. Vitreous changes may comprehend: vitreous hemorrhage, vitreous strands, and vitreous traction. A further classi cation of posterior uveitis depends on the primary site of in ammation, which can identify: retinitis, choroiditis, retinochoroiditis, and chorioretinitis. Posterior pole uveal involvement can be: focal, multifocal, and placoid. Retinal vasculitis can be present. Uveitis might be complicated by anterior and posterior synechiae, which can lead to uveitic glaucoma, cystoid macular oedema, retinal and choroidal neovascularizations, and retinal ischemia.

Non-steroidal anti-inflammatory drugs versus corticosteroids for controlling inflammation after uncomplicated cataract surgery.

Non-steroidal anti-inflammatory drugs versus corticosteroids for controlling inflammation after uncomplicated cataract surgery.

Risk Factors for Secondary Glaucoma in Herpetic Anterior Uveitis

To determine the incidence of elevated intraocular pressure (IOP) and secondary glaucoma in herpetic anterior uveitis (AU), owing to either herpes simplex or varicella zoster virus, by using the Standardization of Uveitis Nomenclature (SUN) criteria, and to identify risk factors for the development of glaucoma. Retrospective observational cohort study. Patients with herpetic AU presenting themselves between 2001 and 2013 at the ophthalmology department of the University Medical Center Groningen were included. Main outcome measures were the incidence of elevated IOP and glaucoma and risk factors for the development of glaucoma. Seventy-three herpetic AU patients were included. Ocular complications most commonly seen during follow-up for uveitis were elevated IOP (75%), keratitis (59%), dry eyes (34%), posterior synechiae (34%), cataract (32%), and glaucoma (15%). Glaucoma patients, in comparison to non-glaucoma patients, had a higher number of IOP peaks during their follow-up for uveitis (P < .001). The majority of patients with elevated IOP (91%) had this already at the start of the uveitis. Nineteen percent of the patients needed glaucoma surgery. Using the SUN criteria, our study confirmed that elevated IOP and secondary glaucoma are major complications in herpetic AU. If an elevated IOP occurred, it was usually already present at the start of a uveitis episode. A risk factor for the development of glaucoma was the number of endured IOP peaks. Future studies are needed to evaluate whether early and prolonged use of antiviral and IOP-lowering medication may prevent glaucoma.

Adalimumab plus Methotrexate for Uveitis in Juvenile Idiopathic Arthritis

BackgroundAdalimumab, a fully human anti–tumor necrosis factor α monoclonal antibody, is effective in the treatment of juvenile idiopathic arthritis (JIA). We tested the efficacy of adalimumab in the treatment of JIA-associated uveitis.MethodsIn this multicenter, double-blind, randomized, placebo-controlled trial, we assessed the efficacy and safety of adalimumab in children and adolescents 2 years of age or older who had active JIA-associated uveitis. Patients who were taking a stable dose of methotrexate were randomly assigned in a 2:1 ratio to receive either adalimumab (at a dose of 20 mg or 40 mg, according to body weight) or placebo, administered subcutaneously every 2 weeks. Patients continued the trial regimen until treatment failure or until 18 months had elapsed. They were followed for up to 2 years after randomization. The primary end point was the time to treatment failure, defined according to a multicomponent intraocular inflammation score that was based on the Standardization of Uveitis Nomenclature criteria.ResultsThe prespecified stopping criteria were met after the enrollment of 90 of 114 patients. We observed 16 treatment failures in 60 patients (27%) in the adalimumab group versus 18 treatment failures in 30 patients (60%) in the placebo group (hazard ratio, 0.25; 95% confidence interval [CI], 0.12 to 0.49; P<0.0001 [the prespecified stopping boundary]). Adverse events were reported more frequently in patients receiving adalimumab than in those receiving placebo (10.07 events per patient-year [95% CI, 9.26 to 10.89] vs. 6.51 events per patient-year [95% CI, 5.26 to 7.77]), as were serious adverse events (0.29 events per patient-year [95% CI, 0.15 to 0.43] vs. 0.19 events per patient-year [95% CI, 0.00 to 0.40]).ConclusionsAdalimumab therapy controlled inflammation and was associated with a lower rate of treatment failure than placebo among children and adolescents with active JIA-associated uveitis who were taking a stable dose of methotrexate. Patients who received adalimumab had a much higher incidence of adverse events and serious adverse events than those who received placebo. (Funded by the NIHR Health Technology Assessment Programme and Arthritis Research UK; SYCAMORE EudraCT number, 2010-021141-41.)

Social Medical Consequences of Uveitis in Childhood and Adolescence: Results of a Pilot Study

Background Chronic illness - such as uveitis - may lead to socio-economic consequences, and poorer quality of life. Objective To study the influence of uveitis on quality of life and professional development. Material and Methods Prospective evaluation of juvenile patients with uveitis (age < 18 years, classification of uveitis as in Standardization of Uveitis Nomenclature [SUN] criteria, duration of disease ≥ 12 months) seen in a tertiary referral centre. Influence of uveitis on quality of life, recreational activities, educational training and development of severe disability were analysed. Results were correlated to clinical data, and analysed by descriptive statistics. Results 39 patients of 49 (80 %) had anterior uveitis (69.4 % in both eyes), of which 34 (69 %) presented with associated systemic disease (juvenile idiopathic arthritis, n = 28). 33 (80 %) had developed complications caused by uveitis. 15 patients (31 %) had undergone ophthalmic surgery due to these complications. Many patients were treated with conventional (n = 37; 76 %) and biological (n = 18; 37 %) disease-modifying antirheumatic drugs (DMARDs). In 41 % of the cases, quality of life and recreational time (23 %) were markedly reduced. Sick leave from pre-school or school because of uveitis (mean 12.5 days/year; 15 % > 20 days) was recorded in 32 patients (65 %). Uveitis negatively influenced school education in 29 %, and resulted in the necessity to repeat class (n = 3) or to change school (n = 3). Severe disability due to uveitis was found in 30 % of the patients. Conclusions Uveitis in childhood and adolescence may have a substantial impact on social and professional perspectives. Uveitis may result in severe disability even in early childhood.

Ten-year retrospective review of outcomes following phacoemulsification with intraocular lens implantation in patients with pre-existing uveitis

ObjectiveThe aim of this study was to evaluate the visual outcome, uveitis control, and complications following cataract surgery for intraocular lens (IOL) implantation in patients with a known diagnosis of uveitis. DesignThe study was a retrospective interventional case series. ParticipantsWe reviewed 98 patients (137 eyes) with adult uveitis undergoing cataract surgery with foldable acrylic posterior chamber IOL implantation between 2003 and 2013 in 2 uveitis practices. MethodsBest-corrected visual acuity (BCVA) and uveitis grade (Standardized Uveitis Nomenclature criteria) were measured at 1 month preoperatively, at postoperative week 1, and at postoperative months 1, 6, and 12. The main outcome measures were mean change in postoperative BCVA, uveitis grade, and complications. ResultsOf the eyes studied, 84% had grade 0–0.5 anterior uveitis at postoperative week 1 and maintained uveitis control (77% grade 0; 19% grade 0.5 anterior uveitis) at 1 year postoperatively. None of the patients had active intermediate or posterior uveitis at any time point. Mean BCVA improved from 0.71 ± 0.38 logMAR preoperatively to 0.37 ± 0.36 at 6 months (p < 0.01) and to 0.30 ± 0.25 at 12 months (p = 0.01) postoperatively. Of the study participants, 30% had preoperative complications related to uveitis, including epiretinal membrane (12%), cystoid macular edema (12%), and glaucoma (5.8%); 46% of patients had small pupils as a result of posterior synechiae. Postoperative vision-limiting complications included posterior capsule opacification (18%), epiretinal membrane (9.0%), and cystoid macular edema (8.8%). Of the eyes studied, 5.8% underwent Nd:YAG capsulotomy. ConclusionsCataract surgery with acrylic posterior-chamber IOL implantation is effective at improving visual acuity in patients with uveitis. Uveitis was well controlled in the majority of our study patients for 12 months after cataract surgery. The most frequent vision-limiting postoperative complication was posterior capsule opacification, which was treatable with Nd:YAG capsulotomy.

Pathophysiology of uveitis

SummaryThis talk will overview the pathophysiology of non‐infectious uveitis in relation to recent SUN (standardised uveitis nomenclature) disease classification. The experimental and translational human evidence of autoimmunity and activation of immunity will be discussed. In addition the talk will highlight the pathways and mechanisms of tissue damage that results in sight‐threatening disease. Traditionally, despite active immune regulatory mechanisms operative within the ocular environment, inflammation still occurs. Activated antigen and non‐antigen specific T cells are generated in uveitis. The interplay with innate immunity and in particular cells of myeloid lineage both systemically and within the local environment dictate the severity and extent of pathology we observe. The understanding of immune responses during the uveitis open many avenues to potential novel immunotherapies that not only suppress inflammation but attempt to redress immune balance, tolerance and local homeostasis within ocular tissues.

Signs and symptoms of uveitis

SummaryUveitis can be a sight‐threatening disease. Inflammation of uveal tract can be divided into: anterior, intermediate, posterior, and panuveitis. Blurred vision, ocular pain, photophobia and floaters are some of the symptoms complained by those who are affected by uveitis. The onset of uveitis can be either acute or insidious, bilateral rather than unilateral. Posterior uveitis is usually associated with vitritis. Anterior chamber cells and flare should be graded according to standardized uveitis nomenclature (SUN) working group. Binocular indirect ophthalmoscopy (BIO) score is used to evaluate the severity of vitritis. Vitreous changes may comprehend: vitreous hemorrhage, vitreous strands, and vitreous traction. A further classification of posterior uveitis depends on the primary site of inflammation, which can identify: retinitis, choroiditis, retinochoroiditis, and chorioretinitis. Posterior pole uveal involvement can be: focal, multifocal, and placoid. Retinal vasculitis can be present. Uveitis might be complicated by anterior and posterior synechiae, which can lead to uveitic glaucoma, cystoid macular oedema, retinal and choroidal neovascularizations, and retinal ischemia.

Adalimumab for the treatment of refractory noninfectious paediatric uveitis.

To report the experience of our center with the use of adalimumab (ADA) for the treatment of severe refractory noninfectious paediatric uveitis. The study is a retrospective case series of all paediatric patients with refractory uveitis who were treated with ADA at the Paediatric Uveitis Unit of our center from 2008 to 2015. We present 12 patients (6 Juvenile idiopathic arthritis-associated uveitis, 4 idiopathic panuveitis, 1 early-onset sarcoidosis-associated panuveitis, and 1 intermediate uveitis), with uveitis in 19/24 eyes. Once ADA therapy was started, all the patients presented improved activity according to Standardization of Uveitis Nomenclature (SUN) criteria. Nine out of the 12 patients had structural damage before ADA could be started: cataract (n=4), glaucoma (n=2), cystic macular edema (n=1), exudative retinal detachment (n=1), and optic disk edema (n=5). Visual acuity improved or maintained stable in 17/19 affected eyes, and only 2 eyes decreased its visual acuity because of structural damage, which was already present before ADA therapy. In our experience, ADA presents a good safety profile and is efficacious in the treatment of paediatric patients with different forms of refractory noninfectious uveitis.

The Feasibility of Spectral-Domain Optical Coherence Tomography Grading of Anterior Chamber Inflammation in a Rabbit Model of Anterior Uveitis.

PurposeTo determine the feasibility and accuracy of spectral-domain optical coherence tomography (SD-OCT) based grading of anterior chamber cell, using aqueous sampling as a standard, in a rabbit model of anterior uveitis.MethodsAdult Dutch-belted rabbits were preimmunized with M. tuberculosis (Tb) H37RA antigen, 1 week prior to induction of anterior uveitis with an intracameral injection of Tb antigen. The anterior chamber was imaged with SD-OCT, followed by a slit lamp examination. Two independent, trained graders recorded their estimate of anterior chamber cell count using the Standardization of Uveitis Nomenclature (SUN) scores for each eye prior to performing an anterior chamber tap to determine the aqueous cell density using a hemocytometer. Using the aqueous cell density as a standard, correlation with SD-OCT counts were compared to those with SUN scores.ResultsOverall, SD-OCT correlated well with the hemocytometer counts (Spearman coefficient = 0.53, P < 0.001) compared with SUN grading and hemocytometer counts (Spearman coefficient = 0.02, P = 0.88). The correlation improved to 0.65 (P < 0.001) when we excluded eyes with corneal thickness ≥ 470 μm. Eyes with corneal thickness ≥ 470 μm exhibited the greatest degree of ocular inflammation and corneal opacity.ConclusionsIn our rabbit model, SD-OCT grading of anterior chamber cell correlated significantly better with aqueous cell counts, compared to traditional slit lamp grading. Spectral-domain optical coherence tomography grading of anterior chamber cell may be a good alternative to SUN grading. Although SUN grading remains the clinical gold standard, alternative quantitative methods to assess ocular inflammation could provide insight into disease mechanism and aid in measuring treatment response.

Discrepancies in assessing anterior chamber activity among uveitis specialists.

To evaluate current practices in anterior chamber (AC) inflammation assessment among uveitis specialists. Uveitis specialists were invited to participate in an electronic survey designed to understand their practice in assessing AC inflammation. Sixty-five ophthalmologists participated in the survey. Of them, 69.2% (n=45) reported using the current Standardization of Uveitis Nomenclature (SUN) guidelines of a 1×1-mm slit beam when grading AC cells. Only 38.5% (n=5) reported routinely counting the number of cells. In the management of uveitis, 98.5% (n=64) valued flare assessment, but 84.6% (n=55) did not use laser flare photometry. In total, 36.9% (n=24) agreed that laser flare photometry would change their management, while 16.9% (n=11) did not see its usefulness. The remaining participants were undecided. A number of issues limit the clinical assessment of AC inflammation. Different classifications are still being used despite efforts to standardize practice. While the value of flare is widely recognized, the role of laser flare photometry remains controversial.

Mycophenolate mofetil as an immunomodulator in refractory noninfectious uveitis

To evaluate the efficacy and tolerance of mycophenolate mofetil (MMF) for the treatment of noninfectious uveitis using the methods advocated by the Standardization of Uveitis Nomenclature (SUN) Working Group and to compare this with other studies of immunosuppression in ocular inflammation. Retrospective case series. Patients with noninfectious uveitis, followed at a tertiary Uveitis Service in São Paulo, Brazil, from 2007 to 2014 and receiving oral MMF for a minimum of 6 months, were retrospectively reviewed. After reaching an optimal dose of MMF, patients were evaluated after 6 (T6), 12 (T12), and 24 months (T24). The optimal dose varied for each patient (medium 2.2 g/day, range 1.0-3.0 g/day). The main outcome measures were: 1) success on achieving complete control of inflammation in both eyes and/or oral prednisone dosage reduction to ≤10 mg per day, and 2) the length of time required to reduce oral prednisone to ≤10 mg/day, partial control of ocular inflammation, and side effects. In a cohort of 16 patients with refractory noninfectious uveitis, 67% reached the ideal prednisone dose after 1 year of MMF treatment and 83% after 2 years of MMF treatment. Complete or partial inflammation control was achieved in 43.7% at T12. Two patients (14%) had disease remission after 4.7 years of MMF treatment. Adverse effects were gastrointestinal disturbances, infection, insomnia, and liver function abnormalities at a rate of 0.03 patient-year each. This small retrospective case series is consistent with the literature concerning the high efficacy and moderate tolerability of MMF in noninfectious uveitis. Observation of patients should be continued for at least 1 year to clearly determine MMF efficacy.

A Rayleigh Scatter-Based Ocular Flare Analysis Meter for Flare Photometry of the Anterior Chamber.

Existing flare photometers are based on the Tyndall effect, which requires sophisticated laser photometry. The ocular flare analysis meter (OFAM) is a nonlaser photometer that uses quantitative Rayleigh scatter and absorption from visible light to compute a flare value. This study is designed to correlate OFAM measurements with qualitative measurements of flare in vitro and in vivo. Following validation of the device on artificial anterior chambers containing known protein concentrations, flare readings were obtained from 90 subjects (46 with and 44 without uveitis) in one eye. Subjects were graded by the Standardization of Uveitis Nomenclature (SUN) working group flare scoring system and received the OFAM flare measurements. The OFAM showed linear response in vitro to protein concentrations ranging from 0 to 0.5 mg/ml. In clinical use in subjects ranging from SUN flare scores of 0+ to 2+, OFAM showed statistically significant measurement accuracy (P = 0.0008 of flare 0 versus flare 2; P = 0.031 of flare 0 versus flare 1). Distinction of SUN scores 1 and 2 was borderline significant (P = 0.057). The OFAM photometry correlates with the standard SUN scoring system. This method may provide an objective method to diagnosis and monitor uveitis. Further longitudinal studies are warranted. Currently, ocular flare is assessed qualitatively in most clinical settings. The existing methodology uses only Tyndall effect to measure flare. The OFAM uses an alternate, nonlaser means for measurement of anterior chamber flare by measure of Raleigh scatter. This pilot clinical study suggests that the OFAM device may be useful in measurement of uveitis activity.

Species Differences in the Geometry of the Anterior Segment Differentially Affect Anterior Chamber Cell Scoring Systems in Laboratory Animals

To determine the impact of anterior segment geometry on ocular scoring systems quantifying anterior chamber (AC) cells in humans and 7 common laboratory species. Using normative anterior segment dimensions and novel geometric formulae, ocular section volumes measured by 3 scoring systems; Standardization of Uveitis Nomenclature (SUN), Ocular Services On Demand (OSOD), and OSOD-modified SUN were calculated for each species, respectively. Calculated volumes were applied to each system's AC cell scoring scheme to determine comparative cell density (cells/mm(3)). Cell density values for all laboratory species were normalized to human values and conversion factors derived to create modified scoring schemes, facilitating interspecies comparison with each system, respectively. Differences in anterior segment geometry resulted in marked differences in optical section volume measured. Volumes were smaller in rodents than dogs and cats, but represented a comparatively larger percentage of AC volume. AC cell density (cells/mm(3)) varied between species. Using the SUN and OSOD-modified SUN systems, values in the pig, dog, and cat underestimated human values; values in rodents overestimated human values. Modified normalized scoring systems presented here account for species-related anterior segment geometry and facilitate both intra- and interspecies analysis, as well as translational comparison. Employment of modified AC cell scoring systems that account for species-specific differences in anterior segment anatomy would harmonize findings across species and may be more predictive for determining ocular toxicological consequences in ocular drug and device development programs.

Pathophysiology of uveitis

SummaryThis talk will overview the pathophysiology of non‐infectious uveitis in relation to recent SUN (standardised uveitis nomenclature) disease classification. The experimental and translational human evidence of autoimmunity and activation of immunity will be discussed. In addition the talk will highlight the pathways and mechanisms of tissue damage that results in sight‐threatening disease. Traditionally, despite active immune regulatory mechanisms operative within the ocular environment, inflammation still occurs. Activated antigen and non‐antigen specific T cells are generated in uveitis. The interplay with innate immunity and in particular cells of myeloid lineage both systemically and within the local environment dictate the severity and extent of pathology we observe. The understanding of immune responses during the uveitis open many avenues to potential novel immunotherapies that not only suppress inflammation but attempt to redress immune balance, tolerance and local homeostasis within ocular tissues.

Signs and symptoms of uveitis

SummaryInflammation of uveal tract can be divided into: anterior, intermediate, posterior, and panuveitis. Uveitis can be a sight‐threatening disease. The commonest ocular symptoms are: blurred vision, ocular pain, photophobia and floaters, depending on the type of uveitis. The onset of uveitis can be either acute or insidious, involving one or both eyes. Posterior uveitis is usually associated with vitritis. Anterior chamber cells and flare should be graded according to standardized uveitis nomenclature (SUN) working group. Binocular indirect ophthalmoscopy (BIO) score is used to grade vitreous involvement. Vitreous changes may comprehend: vitreous hemorrhage, vitreous strands, and vitreous traction. A further classification of posterior uveitis depends on the primary site of inflammation, which can identify: retinitis, choroiditis, retinochoroiditis, and chorioretinitis. Posterior pole uveal involvement can be: focal, multifocal, and placoid. Retinal vasculitis can be associated with several sub‐types of posterior uveitis. Uveitis can present several complications such as, anterior and posterior synechiae, which can lead to uveitic glaucoma, cystoid macular oedema, retinal and choroidal neovascularizations, and retinal ischemia.

Uveitis outcome and complication

Anterior uveitis is a well-known threatening comorbid condition of JIA and affects around 10 to 20 % of the patients depending on JIA subtype. A large proportion of children with JIA develop uveitis in the first year of disease and 73 to 90% do so after four years of the arthritis onset. Uveitis can progress into the adulthood and usually occur as “white uveitis” which is not associated with symptoms like redness and pain as opposed to JIA related to the enthesitis subtype that is symptomatic. Factors associated to lower uveitis remission rate are: JIA diagnosis, findings of 1+ or more vitreous cells at presentation and initial visual acuity of 20/200 or worse. The Standardization of Uveitis Nomenclature (SUN) Group took the first step to define outcome measures for uveitis, but it was established just for adults. The Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC) proposed outcome measures for JIA associated uveitis incorporating the Standardization of Uveitis Nomenclature (SUN) criteria in 2011.

Efficacy of high dose methotrexate in pediatric auto-immune uveitis

Methods Retrospective analysis of 46 pediatric patients with uveitis with or without underlying systemic disease treated with MTX at the University Medical Center Groningen (The Netherlands ) between 1993 and 2013. The SUN (Standardization of Uveitis Nomenclature) workinggroup criteria were used for endpoints. Other endpoints included visual outcome, steroid-sparing effect, cumulative dose MTX to disease remission and side effects.

Classification of uveitis

AbstractClassification and standardization of uveitis is important, as it enhances the precision and comparability of clinical research from different centers and assists in the development of a complete picture of the course of the disorders and their response to treatment. Attempts have been made to standardize some aspects of uveitis, and various classification criteria, inflammation grading schema, and outcomes criteria have been described. The most widely used classification of uveitis is the one devised by the International Uveitis Study Group (IUSG) in 1987, based on the anatomical location of the inflammation. This classification includes anterior uveitis (iritis, iridocyclitis, and anterior cyclitis), intermediate uveitis (pars planitis, posterior cyclitis, and hyalitis), posterior uveitis (focal, multifocal, or diffuse choroiditis, chorioretinitis, retinitis, and neuroretinitis) or panuveitis (anterior chamber, vitreous, retina, and choroid). In 2005, the Standardization of Uveitis Nomenclature (SUN) Working Group standardized a grading schema for aspects of intraocular intraocular inflammation, that is, anterior chamber cells, anterior chamber flare, and vitreous haze, was developed. Standardized definitions of outcomes, including reporting visual acuity outcomes, were approved. Today’s uveitis nomenclature has been revised regarding the anatomical location and the grade of inflammation, and supplemented by the inclusion of definitions for onset, duration and course.

Pathophysiology of uveitis

AbstractThis talk will overview the pathophysiology of non‐infectious uveitis in relation to recent SUN (standardised uveitis nomenclature) disease classification. The experimental and translational human evidence of autoimmunity and activation of immunity will be discussed. In addition the talk will highlight the pathways and mechanisms of tissue damage that results in sight‐threatening disease.Traditionally, despite active immune regulatory mechanisms operative within the ocular environment, inflammation still occurs. Activated antigen and non‐antigen specific T cells are generated in uveitis. The interplay with innate immunity and in particular cells of myeloid lineage both systemically and within the local environment dictate the severity and extent of pathology we observe. The understanding of immune responses during the uveitis open many avenues to potential novel immunotherapies that not only suppress inflammation but attempt to redress immune balance, tolerance and local homeostasis within ocular tissues.