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Purpose: The identification of infectious etiologies is important in the management of uveitis. Ocular fluid testing is required, but multiplex testing faces challenges due to the limited volume sampled. The determination of antibody repertoire of aqueous humor (AH) is not possible with conventional assays. We investigated the use of a highly multiplexable serological assay VirScan, a Phage ImmunoPrecipitation Sequencing (PhIP-Seq) library derived from the sequences of more than 200 viruses to determine the antibody composition of AH in patients with uveitis., Design: Prospective, case control study., Methods: We analyzed the paired AH and plasma samples of 11 immunocompetent patients with active polymerase chain reaction-positive cytomegalovirus (CMV) anterior uveitis and the AH of 34 control patients undergoing cataract surgery with no known uveitis in an institutional practice. The samples were tested using VirScan PhIP-Seq, and the entire pan-viral antibody repertoire was determined using peptide tile ranking by normalized counts to identify significant antibodies enrichment against all viruses with human tropism., Results: Significant enrichment of antibodies to Herpesviridae, Picornavirdae, and Paramyxoviridae was detectable in 20 µL of AH samples from patients with CMV uveitis and controls. Patients with CMV uveitis had relative enrichment of anti-CMV antibodies in AH compared with their plasma. Epitope-level mapping identified significant enrichment of antibodies against CMV tegument protein pp150 (P = 1.5e-06) and envelope glycoprotein B (P = .0045) in the AH compared with controls., Conclusions: Our proof-of-concept study not only sheds light on the antibody repertoire of AH but also expands the utility of PhIP-Seq to future studies to detect antibodies in AH in the study of inflammatory eye diseases., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: A significant number of children with noninfectious, chronic anterior uveitis (CAU) fail to respond to conventional therapy; however, successful alternative biologic treatments (ABT) have not been well described. This study aims to review the clinical and treatment characteristics of children with CAU who require ABT., Design: Retrospective, nonrandomized clinical study., Methods: Setting: Tertiary center., Study Population: Children with noninfectious CAU., Observation Procedures: Clinical characteristics, uveitis course, complications, and treatment were compared among patients treated with methotrexate (MTX) monotherapy, conventional TNFα inhibitors (cTNFi), and ABT for >3 months., Main Outcome Measure: Success of ABT (abatacept, tocilizumab, and/or golimumab) in children failing conventional treatment., Results: Of the 52 children with CAU, 75% had juvenile idiopathic arthritis. CAU was controlled in 15 children receiving MTX monotherapy, 28 receiving cTNFi, and 9 receiving ABT (n = 1, abatacept; n = 3, tocilizumab; n = 5, golimumab). Patients in the ABT group had a greater number of total ocular complications per person before ABT than those in the control groups (3.4 vs 0.7 [MTX], P < .001, and 1.5 [cTNFi], P < .001, respectively). In all 9 children on ABT, treatment led to control of CAU and topical glucocorticoids tapered to ≤2 drops/d with no new ocular complications., Conclusions: In this study, alternative biologics (abatacept, golimumab, and tocilizumab) were useful for treating CAU in children who fail MTX and cTNFi therapy. Patients who were controlled on ABT had more disease activity, ocular complications, and anti-cTNFi neutralizing antibodies (before ABT) than those managed with conventional therapy. Larger studies are required to confirm these findings., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the feasibility of anterior segment optical coherence tomography (AS-OCT) for measuring anterior chamber (AC) cells in children with uveitis and to compare different AS-OCT acquisition modes., Design: Validity and reliability analysis., Methods: We enrolled children younger than 18 years who had uveitis involving the anterior segment and children without eye disease as controls. All underwent clinical grading of AC cells. AC images of each eye were obtained using the Optovue Avanti RTVue XR AS-OCT. Two acquisition modes were used: a single cross-sectional line scan and an 8-line radial scan in an asterisk pattern. Two independent, masked graders counted cells manually on AS-OCT images. Rater agreement was assessed using intraclass correlation (ICC)., Results: Included were 30 children (59 eyes) with uveitis (median age 13.0 years, range 3-17 years) and 20 control children (40 eyes, median age 10.5 years, range 4-17 years). The number of eyes assigned each clinical grade of cells were as follows: none, 32 (54%); 0.5+, 12 (20.3%); 1+, 5 (8.5%); 2+, 8 (13.6%); 3+, 2 (3.4%). ICC of graders for line and radial scan protocols were 0.87 and 0.90. There was no significant difference between acquisition modes for pooled grader results (95% CI for difference: -0.04 to 0.14). ICC of cell counts between line and radial scan protocols was 0.85 (95% CI: 0.69-0.90). No control eyes had cells on AS-OCT images., Conclusions: Quantification of AC cell in children with uveitis is feasible with AS-OCT and has excellent reliability between different graders and acquisition modes., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Purpose: To examine the clinical course and outcome in children with idiopathic chronic anterior uveitis (iCAU), and to compare the results with those of age-matched children with juvenile idiopathic arthritis-associated uveitis (JIA-U)., Design: Retrospective cohort study., Methods: Data regarding ocular complications, visual acuity, and systemic treatment were retrospectively collected for 2 patient groups that were matched regarding age and year of uveitis diagnosis. Outcome was evaluated using survival analysis., Results: The iCAU and JIA-U groups included 48 patients with 83 affected eyes and 48 patients with 73 affected eyes, respectively. Multivariate analyses showed that iCAU was associated with a higher prevalence of posterior synechiae (adjusted hazard rate [aHR] = 3.63; P < .001) and cataract surgery (aHR = 2.90; P = .006). Baseline visual acuity was worse in the iCAU group compared to the JIA-U group (20/25 vs 20/20, respectively; P < .001), but improved in the iCAU group after 5 years (20/20 vs 20/20, respectively; P = .052). At the 5-year follow-up, the younger children with iCAU (≤8 years of age at diagnosis) had a higher prevalence of posterior synechiae (aHR = 2.56; P = .007), secondary glaucoma (aHR = 16.0; P = .020), and cataract surgery (aHR = 4.79; P = .004) compared to older children with iCAU (≥9 years at diagnosis)., Conclusions: Vision-threatening ocular complications are more common in children with iCAU compared to children with JIA-U, particularly in cases in which the onset of uveitis occurred at ≤8 years of age. However, the long-term vision of these children can be improved with adequate treatment., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Cytomegalovirus (CMV) anterior uveitis is the most common form of ocular manifestation of CMV in immunocompetent individuals. The difficulty in diagnosing CMV anterior uveitis may delay adequate treatment and affect outcomes. We sought to review systemically the overall clinical characteristics and compare treatment outcomes in CMV anterior uveitis and endotheliitis. A literature search was performed, and studies describing clinical characteristics, treatment regimens, and outcomes that included more than 5 treated eyes were included. In these 23 studies, acute CMV anterior uveitis commonly presented with high intraocular pressure (95.31%, 95% CI 90.45-98.60) and mild anterior chamber inflammation (cells >2+ = 3.18%, 95% CI 0.21-0.54). About two-thirds of CMV endotheliitis cases presented with high intraocular pressure and coin-shaped corneal lesions. Acute CMV anterior uveitis showed good clinical response to topical 0.15% ganciclovir (GCV) gel or oral valganciclovir (VGCV) (90%, 95% CI 74-100% and 95%, 95% CI 88-100%, respectively). For chronic CMV anterior uveitis, both topical GCV and oral VGCV yielded comparable results. Topical 0.5-2% GCV or a combination of topical and oral VGCV for CMV endotheliitis both resulted in good clinical response. Recurrence of inflammation was common after cessation of maintenance therapy. Overall, topical GCV resulted in an optimal outcome for CMV anterior uveitis. Escalated concentration and frequency of usage are needed for chronic CMV anterior uveitis and endotheliitis. Adequate induction and maintenance phases of anti-CMV treatment seem necessary to prevent recurrences., (Copyright © 2021. Published by Elsevier Inc.)

Purpose: Spondyloarthritis (SpA) is a group of diseases with overlapping skeletal and extra-articular features. Acute anterior uveitis (AAU) is the most common extra-articular manifestation of SpA. The relation between AAU and SpA is well defined in the current literature. Our study aims to analyze the frequency and factors associated with AAU in different forms of SpA in a large nationwide cohort of Turkish SpA patients., Design: Retrospective cohort study., Methods: The data were obtained from the TReasure database, which compiles data from records of the web-based Rheumatoid Arthritis (RA) and SpA patients treated with biological disease-modifying anti-rheumatismal drugs from different regions of Turkey. The clinical characteristics of SpA and uveitis are recorded., Results: Data of the 4,297 SpA patients were included in the study. Overall, 475 of 4,297 patients (11.0%) had experienced 1 or more episodes of uveitis. SpA patients with older age (P < .001), a smoking history (P = .004), delayed diagnosis (P = .001), longer disease duration (P < .001), arthritis (P < .001), positive HLA-B27 (P < .001), a family history of SpA (P < .001), and radiographic damage (presence of sacroiliitis, syndesmophytes, bamboo spine, hip involvement) (P < .001 for all) more commonly had uveitis. On the other hand, uveitis was less prevalent in patients with psoriasis and psoriatic arthritis (P < .001 for both)., Conclusion: Uveitis may be the key feature leading to SpA diagnosis. Patients with radiographic damage and long disease duration have an increased risk for uveitis in both male and female SpA patients. Patients with uveitis should be referred to a rheumatologist for a thorough evaluation of SpA., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: To determine classification criteria for varicella zoster virus (VZV) anterior uveitis., Design: Machine learning of cases with VZV anterior uveitis and 8 other anterior uveitides., Methods: Cases of anterior uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the anterior uveitides. The resulting criteria were evaluated on the validation set., Results: One thousand eighty-three cases of anterior uveitides, including 123 cases of VZV anterior uveitis, were evaluated by machine learning. The overall accuracy for anterior uveitides was 97.5% in the training set and 96.7% in the validation set (95% confidence interval 92.4, 98.6). Key criteria for VZV anterior uveitis included unilateral anterior uveitis with either (1) positive aqueous humor polymerase chain reaction assay for VZV; (2) sectoral iris atrophy in a patient ≥60 years of age; or (3) concurrent or recent dermatomal herpes zoster. The misclassification rates for VZV anterior uveitis were 0.9% in the training set and 0% in the validation set, respectively., Conclusions: The criteria for VZV anterior uveitis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to determine classification criteria for herpes simplex virus (HSV) anterior uveitis DESIGN: Machine learning of cases with HSV anterior uveitis and 8 other anterior uveitides., Methods: Cases of anterior uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used in the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the anterior uveitides. The resulting criteria were evaluated in the validation set., Results: A total of 1,083 cases of anterior uveitides, including 101 cases of HSV anterior uveitis, were evaluated by machine learning. The overall accuracy for anterior uveitides was 97.5% in the training set and 96.7% in the validation set (95% confidence interval: 92.4-98.6). Key criteria for HSV anterior uveitis included unilateral anterior uveitis with either 1) positive aqueous humor polymerase chain reaction assay for HSV; 2) sectoral iris atrophy in a patient ≤50 years old; or 3) HSV keratitis. The misclassification rates for HSV anterior uveitis were 8.3% in the training set and 17% in the validation set., Conclusions: The criteria for HSV anterior uveitis had a reasonably low misclassification rate and appeared to perform well enough for use in clinical and translational research., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: To determine classification criteria for cytomegalovirus (CMV) anterior uveitis., Design: Machine learning of cases with CMV anterior uveitis and 8 other anterior uveitides., Methods: Cases of anterior uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the anterior uveitides. The resulting criteria were evaluated on the validation set., Results: One thousand eighty-three cases of anterior uveitides, including 89 cases of CMV anterior uveitis, were evaluated by machine learning. The overall accuracy for anterior uveitides was 97.5% in the training set and 96.7% in the validation set (95% confidence interval 92.4, 98.6). Key criteria for CMV anterior uveitis included unilateral anterior uveitis with a positive aqueous humor polymerase chain reaction assay for CMV. No clinical features reliably diagnosed CMV anterior uveitis. The misclassification rates for CMV anterior uveitis were 1.3% in the training set and 0% in the validation set., Conclusions: The criteria for CMV anterior uveitis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: To determine classification criteria for juvenile idiopathic arthritis (JIA)-associated chronic anterior uveitis (CAU)., Design: Machine learning of cases with JIA CAU and 8 other anterior uveitides., Methods: Cases of anterior uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the anterior uveitides. The resulting criteria were evaluated on the validation set., Results: One thousand eighty-three cases of anterior uveitides, including 202 cases of JIA CAU, were evaluated by machine learning. The overall accuracy for anterior uveitides was 97.5% in the training set and 96.7% in the validation set (95% confidence interval 92.4, 98.6). Key criteria for JIA CAU included (1) chronic anterior uveitis (or, if newly diagnosed, insidious onset) and (2) JIA, except for the systemic, rheumatoid factor-positive polyarthritis, and enthesitis-related arthritis variants. The misclassification rates for JIA CAU were 2.4% in the training set and 0% in the validation set., Conclusions: The criteria for JIA CAU had a low misclassification rate and seemed to perform well enough for use in clinical and translational research., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: To determine distinguishing features of the clinical characteristics of anterior uveitis (AU) caused by herpes simplex virus (HSV), varicella-zoster virus (VZV), and cytomegalovirus (CMV)., Design: Retrospective, multicenter case series., Methods: Consecutive patients with herpetic AU examined at 11 tertiary centers in Japan between January 2012 and December 2017 and who were followed for ≥3 months were evaluated. Diagnosis was made by polymerase chain reaction (PCR) for HSV, VZV, or CMV in the aqueous humor, or classical signs of herpes zoster ophthalmicus., Results: This study enrolled 259 herpetic AU patients, including PCR-proven HSV-AU (30 patients), VZV-AU (50), and CMV-AU (147), and herpes zoster ophthalmicus (32). All HSV-AU and VZV-AU patients were unilateral, while 3% of CMV-AU patients were bilateral. Most HSV-AU and VZV-AU patients were sudden onset with an acute clinical course, while CMV-AU had a more insidious onset and chronic course. There were no significant differences for all surveyed symptoms, signs, and complications between HSV-AU and VZV-AU. However, significant differences were detected for many items between CMV-AU and the other two herpetic AU types. Ocular hyperemia and pain, blurring of vision, ciliary injection, medium-to-large keratic precipitates (KPs), cells and flare in the anterior chamber, and posterior synechia significantly more often occurred in HSV-AU and VZV-AU vs CMV-AU. In contrast, small KPs, coin-shaped KPs, diffuse iris atrophy, elevated intraocular pressure, and glaucoma surgery were significantly more frequent in CMV-AU vs HSV-AU and VZV-AU., Conclusion: This multicenter, retrospective study identified distinguishing features of HSV-AU, VZV-AU, and CMV-AU., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the safety and efficacy of dexamethasone phosphate ophthalmic solution (EGP-437) delivered by a transscleral iontophoresis delivery system (EyeGate II) compared to that of topical prednisolone acetate 1% (PA 1%) in subjects with noninfectious anterior uveitis., Design: Prospective, randomized, double-masked, parallel group, noninferiority clinical trial., Methods: A total of 193 subjects with active noninfectious anterior uveitis (anterior chamber [AC] cell count ≥11 cells) were randomized to EGP-437 delivered by iontophoresis (days 0 and 7) or self-administered PA 1% daily (tapered schedule, days 0-28). Masking was maintained with placebo iontophoresis or eye drops. The primary efficacy endpoint was the proportion of subjects with an AC cell count of zero on day 14. Noninferiority of EGP-437 was defined if the lower limit of the confidence interval (CI) for the difference (EGP-437 minus PA 1%) was less than -10%., Results: At day 14, 32 of 96 EGP-437 subjects (33.3%) and 32 of 97 PA 1% subjects (33.0%) had an AC cell count of zero (difference, 0.34; 95% CI, -12.94 to 13.63; P = 0.064). Efficacy trended better with EGP-437 among patients with more severe baseline uveitis (AC cell count >25). Safety and tolerability were good with both treatments. EGP-437 subjects experienced fewer IOP elevations ≥6 mm Hg versus PA 1% subjects (13 vs 24 incidents, respectively, through day 28)., Conclusions: Despite clinically similar response rates, statistical noninferiority of EGP-437 versus a tapered regimen of PA 1% was not achieved. Numerical trends suggesting fewer IOP elevations with EGP-437, similar efficacy overall, and possibly better efficacy in more severe disease warrant further study., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: To report the characteristics of cytomegalovirus anterior uveitis (CMV AU) and the comparative response to 2 types of antiviral induction treatments., Design: Retrospective, consecutive case series., Methods: Consecutive immunocompetent patients with polymerase chain reaction-positive CMV AU were included. For each patient, best-corrected visual acuity (BCVA), intraocular pressure (IOP), clinical characteristics at baseline and latest visit, and number of relapses were recorded. All patients received an induction dose of intravenous (IV) ganciclovir or oral valganciclovir and a maintenance dose of oral valganciclovir., Results: Thirty-six eyes of 35 patients were included. Mean age at diagnosis was 55.5 years. Mean follow-up was 4.13 years. Posner-Schlossman and chronic nonspecific AU were observed in 69.4% and 30.6% of cases, respectively. We did not observe any case of Fuchs uveitis or endotheliitis. At baseline, mean BCVA was 20/25 and mean IOP was 29.19 mm Hg. Keratic precipitates and iris atrophy were seen in 91.4% and 25.7% of cases. Induction therapy consisted of oral valganciclovir and IV ganciclovir in 40% and 60% of cases. A total of 94.2% of patients responded to the first line of therapy. Recurrence was reported in 73.5% of cases. Glaucoma surgery was necessary in 25.7% of cases. Early initiation of antiviral therapy (≤700 days) seemed to decrease the recourse to glaucoma surgery. Both IV and oral induction treatments seemed similar in terms of BCVA changes and occurrence of relapses., Conclusions: Characteristics of CMV AU seem to show specificities in this French cohort. Early initiation of antiviral therapy seems to reduce the severity of glaucoma., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To analyze occurrence, risk factors, and course of ocular hypotony (OH) in juvenile idiopathic arthritis-associated uveitis (JIAU)., Design: Cohort study., Methods: Epidemiologic and ophthalmologic data at baseline and during follow-up of JIAU patients with and without ocular hypotony were evaluated., Results: OH developed in 57 of the 365 JIAU patients during the follow-up (mean 4.5 ± 3.5 years). In 40 patients with follow-up ≥12 months, OH was unrelated to previous ocular surgery: risk factors at baseline (univariate logistic regression analysis) included longer total duration of uveitis (odds ratio [OR] 1.13, P < .001), bilateral uveitis (OR 3.51, P = .009), low visual acuity (OR 5.1, P = .001), high laser-flare (LF) values (OR 1.74, P = .01), and presence of posterior synechiae (OR 3.28, P = .004). Increased anterior chamber (AC) cell and LF values were observed within 3 months prior to onset of transient (≤3 months; 37.5%) or persistent OH (>3 months; 62.5%). AC cell and LF values decreased within 3 months after onset of transient OH, while LF levels remained elevated ≥12 months in persistent OH. Optic disc edema and epiretinal membrane formation was found more frequently after OH onset., Conclusions: OH was observed in 15.6% of JIAU patients. Longer total uveitis duration, bilateral uveitis, low visual acuity, high AC flare and LF grades, and presence of posterior synechiae at baseline were risk factors for subsequent OH. Burden of OH might be improved with immunosuppression., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: To describe risk factors for hypotony in patients with juvenile idiopathic arthritis (JIA)-associated uveitis., Design: Retrospective cohort study., Methods: All patients with JIA-associated uveitis (N = 108; affected eyes = 196) evaluated and followed at the Wilmer Eye Institute from July 1984 through June 2014 were included in this study. Prevalence and incidence of hypotony (intraocular pressure [IOP] <5 mm Hg) and low IOP (5 mm Hg ≤ IOP < 8 mm Hg) and risk factors for developing hypotony were analyzed., Results: At presentation, 9.3% of patients (7.1% of affected eyes) had hypotony. During a median follow-up of 5.3 years, the rate of developing hypotony and low IOP were 0.04 per eye-year (/EY; 95% confidence interval [CI]: 0.02/EY, 0.05/EY) and 0.06/EY (95% CI: 0.04/EY, 0.08/EY), respectively. Risk factors for development of hypotony during follow-up appeared to be associated with more severe uveitic disease, such as the presence of panuveitis (adjusted hazard ratio [aHR], 43.1; P = .004), anterior chamber cells or flare ≥ 3+ (aHR, 25.6, P < .001), posterior synechiae (aHR, 5.9, P = .02), and the use of oral corticosteroid (aHR 28.9; P = .003) at the presenting examination. Receiving immunosuppressive drug therapy at the time of presentation was associated with a lower risk of development of hypotony (aHR, 0.02; P = .002)., Conclusions: Hypotony affects a small but significant proportion of patients with JIA-associated uveitis and is associated with signs of active and severe uveitis. Immunosuppression was associated with significantly lower risk of hypotony, suggesting that aggressive control of the inflammation may reduce risk of hypotony in JIA-associated uveitis., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To describe nonglaucomatous retinal nerve fiber layer (RNFL) defects in patients with Behçet uveitis., Design: Cross-sectional study and observational case series., Methods: We reviewed the clinical photographs of patients with Behçet uveitis (n = 259), ocular toxoplasmosis (n = 120), and multiple sclerosis (MS)-associated uveitis (n = 40) for the presence of localized RNFL defects. Behçet patients with localized RNFL defects were invited to participate in a prospective evaluation including standard automated perimetry, spectral-domain optical coherence tomography (SD OCT), and RNFL thickness analysis. Main outcome measures were the prevalence of localized RNFL defects, perimetric and SD OCT findings, and RNFL thickness analysis., Results: Sixty-two patients with Behçet uveitis (24%) had localized RNFL defect(s) without any visible scar. Twenty patients (17%) with ocular toxoplasmosis had a localized RNFL defect associated with a retinochoroidal scar in all. None of the MS patients had a localized RNFL defect. Of the 19 patients (24 eyes) with RNFL defect associated with Behçet uveitis who returned for follow-up, there was a corollary thinning on SD OCT B-scan in all and a corresponding visual field defect in 83%. RNFL thickness analysis was within normal limits in 54%, but revealed thinning in the thickness profile scale in 46% and in the pie chart in 21%., Conclusion: In Behçet uveitis, localized RNFL defects may be caused by microvascular ischemia at the optic nerve head and/or at the posterior pole and may serve as a helpful ocular diagnostic clue and an indicator of posterior pole involvement, a risk factor for poor visual prognosis., (Copyright © 2015 Elsevier Inc. All rights reserved.)

A 72-year-old man presented with signs on uveítis. Biopsy of an iris lesion established the diagnosis of uveal MALT lymphoma with presumed choroidal involvement., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: To describe the clinical presentation of uveitis with coincident onset of raised and indurated tattooed skin., Design: Case series., Methods: Seven consecutive patients were evaluated at a tertiary ophthalmologic facility with coincident uveitis and cutaneous tattoo induration over an 18-month period. All subjects underwent complete ophthalmic examination and a focused systemic medical evaluation including serologic testing and imaging studies. Two participants underwent biopsy of their tattoos. The patients' clinical courses and responses to treatment over a follow-up period of 1-20 months are reported (mean follow-up = 9 months). Main outcome measures included degree of intraocular inflammation, ocular complications, visual acuity, clinically observable tattooed skin changes, and biopsy results., Results: Five of 7 patients had bilateral nongranulomatous anterior uveitis: 4 with chronic and 1 with recurrent disease. The remaining 2 patients had bilateral chronic granulomatous panuveitis. Biopsies of raised and indurated tattoos were performed in 2 patients and demonstrated noncaseating granulomatous inflammation surrounding tattoo ink in the dermis. The skin changes resolved in all patients, with a faster response noted in those treated with high-dose oral prednisone for intraocular inflammation. Five patients subsequently experienced recurrent flares of intraocular inflammation in conjunction with the recurrence of raised and indurated tattoos., Conclusions: These cases represent a subset of patients in whom skin tattooing may have incited an immune response leading to simultaneous inflammation of the eyes and tattooed skin., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: To investigate effects of enoxaparin on postoperative inflammation in patients with moderate nonproliferative diabetic retinopathy (NPDR) and nuclear cataract., Design: Prospective masked controlled trial., Methods: setting: Institutional practice. study population: The study included 51 eyes of 51 diabetes mellitus (DM) patients with moderate NPDR and grade 2-3 nuclear cataracts. Group 1 included randomly selected patients who received enoxaparin in balanced salt solution (BSS) during cataract surgery, while standard BSS was used in Group 2. observation procedure: Patients were followed up 1 day, 1 week, and 1 and 2 months after the surgery. main outcome measures: Anterior chamber cell and flare., Results: Postoperatively, 20 patients from Group 1 and 4 patients in Group 2 had less than a mean of 10 cells at day 1 (P = .012). At the first week, 20 patients from Group 1 and 10 patients from Group 2 had less than a mean of 10 cells (P = .004). When compared for flare, 20 patients from Group 1 and 8 patients from Group 2 had a flare between 0 and +1 at postoperative day 1 (P < .001). By the end of postoperative first week, 24 patients from Group 1and 18 patients from Group 2 had a flare between 0 and +1 (P = .012). There was no difference between groups in terms of postoperative inflammation at the first and second months after the surgery., Conclusion: The results of the study suggest that enoxaparin added into the infusion fluid may reduce postoperative inflammation in patients with DM, and this result supports the anti-inflammatory effect of enoxaparin., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Herpes zoster ophthalmicus (HZO) is a common viral infectious disorder affecting the ophthalmic division of the trigeminal nerve. A small subset of HZO patients present with the ophthalmic symptoms, but without an accompanied rash, a condition described as Herpes zoster sine herpete. Although HZO is well known to be associated with other central nervous system abnormalities, encephalitis and cerebral infarction are atypical and uncommon. We report an unusual case of presumed unilateral Herpes zoster ophthalmicus sine herpete that presented with trigeminal pain and uveitis and then progressed to encephalitis and bilateral cerebral infarctions despite treatment with acyclovir and corticosteroids. The diagnosis of HZV was confirmed by polymerase chain reaction testing on the cerebrospinal fluid., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: To describe an approach to diagnosing the uveitides, a collection of about 30 separate diseases characterized by intraocular inflammation., Design: Perspective., Methods: Integration of clinical approach with a more formal, informatics-derived approach to characterization and a Bayesian approach to laboratory testing., Results: The patient's uveitis is characterized along several dimensions: course, laterality, anatomic location of the inflammation, morphology, presence of active infection, and the host (age, presence of a systemic disease). Posterior uveitis can be characterized further by whether it is primarily a retinitis, choroiditis, or retinal vasculitis; by whether it is paucifocal or multifocal; and by the morphology of the lesions. This characterization narrows the differential diagnosis to 1 or, at most, a few diseases. Laboratory screening (ie, testing all patients) should be reserved for those diseases that can present as any type of uveitis, whereas targeted testing (ie, testing a subset with specific features) is used selectively. Laboratory testing should be used to identify an infection (which will alter therapy) or a systemic disease that will affect the patient's health. A uveitis that is not one of the established diagnoses is designated as "undifferentiated" with the course, laterality, and anatomic location (eg, undifferentiated bilateral chronic anterior uveitis). We avoid the term "idiopathic" uveitis as most identified noninfectious uveitic diseases are idiopathic, and most systemic diseases associated with uveitis also are idiopathic (eg, juvenile idiopathic arthritis)., Conclusion: This approach should lead to the correct diagnosis of the specific uveitic disease in the large majority of cases without overuse of laboratory testing., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: To determine the value of anterior chamber paracentesis with polymerase chain reaction (PCR) in patients with anterior uveitis., Design: Retrospective observational case series., Setting: Bascom Palmer Eye Institute., Patient Population: Fifty-three patients with a diagnosis of anterior uveitis who underwent anterior chamber paracentesis with PCR., Observation Procedures: Anterior chamber paracentesis with PCR of aqueous fluid., Main Outcome Measure: Diagnostic utility and frequency of management change based on anterior chamber paracentesis and PCR., Results: There were 15 of 53 (28%) acute and 35 of 53 (65%) chronic anterior uveitis patients. PCR positivity of herpes simplex virus, cytomegalovirus, varicella zoster virus, and Epstein-Barr virus in our population were 4 of 53 (8%), 1 of 47 (2%), 1 of 35 (3%), and 1 of 18 (6%). Overall, 7 of 53 patients (13%) had a change in management because of PCR results from anterior chamber paracentesis. Four patients encountered paracentesis complications (4/53, 7.5%), 1 with long-term sequelae., Conclusions: Anterior chamber paracentesis with PCR had a relatively low diagnostic utility and resulted in few management changes in patients with suspected infectious anterior uveitis., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: To determine the prevalence of inflammatory back pain in an anterior uveitis cohort., Design: Retrospective cohort study., Methods: Patients with anterior uveitis were recruited from the clinic of an ophthalmologist to complete a survey between March and December 2008. Patients were classified with inflammatory back pain if they had ≥2 positive responses to 4 validated inflammatory back pain questions: presence of morning stiffness >30 minutes in duration; improvement in back pain with exercise but not with rest; awakening from back pain during the second half of the night only; and presence of alternating buttock pain. Disease activity was assessed using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). The impact of disease on quality of life was measured using the EuroQOL (EQ-5D) questionnaire. Twenty-five patients underwent further rheumatologic examination., Results: One hundred forty-one of 167 patients (84.4%) completed the survey. Sixty-six of 141 patients (46.8%) were classified to have inflammatory back pain. Mean BASDAI (4.2, SD 2.41) and EQ-5D scores (0.73, SD 0.21) were lower than patients with no inflammatory back pain (0.82, SD 0.16, P = .0048). In the subgroup that underwent rheumatologic assessment, a classification of inflammatory back pain was 92% sensitive and 67% specific for a diagnosis of inflammatory back pain., Conclusions: The prevalence of inflammatory back pain in a cohort of anterior uveitis patients was found to be 46.8%. Patients with inflammatory back pain had worse quality of life than those without. Ophthalmologists may use these questions on back pain to select patients classified to have inflammatory back pain to refer for early rheumatologic assessment., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: To demonstrate the clinical significance of owl eye morphologic features observed by in vivo laser confocal microscopy in patients with cytomegalovirus (CMV) corneal endotheliitis., Design: Observational case series., Methods: participants: Six eyes of 6 patients (6 men; mean age, 73.3 years) with cytomegalovirus corneal endotheliitis diagnosed by clinical manifestations together with polymerase chain reaction from aqueous humor samples. intervention: All patients were examined by slit-lamp biomicroscopy and in vivo laser confocal microscopy. main outcome measures: Clinical manifestations were summarized by reviewing medical records. Selected confocal images of corneal layers were evaluated qualitatively for shape and degree of light reflection of abnormal cells and deposits., Results: All patients had long histories of anterior uveitis with intraocular pressure elevation, corneal edema with keratic precipitates, and decrease of endothelial cell densities. Coin-shaped lesions were observed by slit lamp only in 1 patient at the first visit and in 2 additional patients at subsequent follow-up. In all patients, confocal microscopy demonstrated reduced subepithelial nerves, subepithelial opacity, increased reflectivity of keratocytes, highly reflective dots, and needle-shaped bodies. Owl eye morphologic features were observed consistently in all patients at the initial visit, and highly reflective round bodies were detected in 5 patients; most notably, these confocal features were reversible after resolution of endotheliitis., Conclusions: Owl eye morphologic features and highly reflective round bodies observed by confocal microscopy may be useful as an adjunct for the noninvasive diagnosis of cytomegalovirus corneal endotheliitis. Reversibility of these features after resolution of endotheliitis may be useful for monitoring the therapeutic effects without multiple anterior chamber tap., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Purpose: To describe immune ring (IR) formation as a manifestation of cytomegalovirus (CMV) endotheliitis., Design: Retrospective observational case series., Methods: Review of all consecutive CMV anterior uveitis and endotheliitis patients seen at the Singapore National Eye Centre for the occurrence of an IR. CMV infection was diagnosed by polymerase chain reaction analysis of the aqueous., Results: None of the 72 eyes with CMV anterior uveitis had an IR formation. Four episodes of IR formation were seen in 3 eyes (14.3%) of 21 cases of CMV-positive endotheliitis. All were unilateral and all 3 patients were human immunodeficiency virus negative middle-aged Chinese males. The IR developed 2 to 7 months after occurrence of the endotheliitis and the aqueous was positive for CMV during 2 of the episodes of IR formation. In 2 eyes, the IR occurred at the completion of a course of systemic ganciclovir when the aqueous was negative for CMV. The immune rings resolved with combination therapy of topical prednisolone acetate 0.12% and ganciclovir. Patient 1 had a recurrence of the IR 4 months after stopping treatment but again resolved following treatment with ganciclovir and topical corticosteroids., Conclusion: Corneal immune rings can occur as a result of CMV infection. Hence CMV infection may have to be considered in such cases., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the efficacy of methotrexate (MTX) and the effect of its withdrawal on relapse rate of uveitis associated with juvenile idiopathic arthritis (JIA)., Design: Retrospective case series., Methods: Data of 22 pediatric JIA patients who were being treated with MTX for active uveitis were studied retrospectively. Relapse rate after the withdrawal of MTX was established. Anterior chamber (AC) inflammation, topical steroid use during the first year of MTX treatment, and associations of relapses after the withdrawal were evaluated statistically. Duration of MTX treatment and its withdrawal was determined individually in collaboration with a rheumatologist with an intention to continue the treatment for at least 1 year and to withdraw in case of inactivity of uveitis and arthritis. Inactivity of uveitis was defined as the presence of ≤0.5+ cells in the AC., Results: Eighteen patients (18/22; 82%) showed improvement of their uveitis with a significant decrease in activity of AC inflammation after a minimal period of 3 months of MTX treatment. A topical steroid-sparing effect was observed when MTX was administered for a period of 3 to 9 months. MTX was discontinued because of inactive uveitis in 13 patients. In 9 patients (8/13; 69%) a relapse of uveitis was observed after a mean time of 7.5 months (± SD 7.3). Six patients (6/13; 46%) had a relapse within the first year after the withdrawal. Relapse-free survival after withdrawal of MTX was significantly longer in patients who had been treated with MTX for more than 3 years (P = .009), children who were older than 8 years at the moment of withdrawal (P = .003), and patients who had an inactivity of uveitis of longer than 2 years before withdrawal of MTX (P = .033). Longer inactivity under MTX therapy was independently protective for relapses after the withdrawal (hazard ratio = 0.07; 95% confidence interval 0.01-0.86; P = .038), which means that 1-year increase of duration of inactive uveitis before the withdrawal of MTX results in a decrease of hazard for new relapse of 93%., Conclusions: A high number of patients with inactive uveitis relapse quickly after the withdrawal of MTX. Our results suggest that a longer period of inactivity prior to withdrawal and a longer treatment period with MTX reduce the chance of relapse after withdrawal., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: To report a case and management of uveitis-glaucoma-hyphema (UGH) syndrome and corneal decompensation associated with cosmetic iris implants., Design: Interventional case report., Settings: Department of Ophthalmology, University of Minnesota School of Medicine., Patients: A 29-year-old man presented with bilateral redness, severe pain, photophobia, and reduction in visual acuity. Examination revealed intraocular pressure (IOP) of 38 mm Hg and 40 mm Hg right and left eye respectively, bilateral conjunctival injection 3+, epithelial corneal edema, microhyphema, cells 3+ to 4+ and flare 2+, bilateral cosmetic iris implants, and surgical peripheral iridectomies. The patient was diagnosed with UGH syndrome and corneal decompensation associated with cosmetic iris implants., Intervention: Immediate medical management of inflammation and elevated IOP, anterior segment imaging, specular microscopy, Humphrey 24-2 visual field (VF) testing; following failed conservative management, combined trabeculectomy and removal of the iris implants., Main Outcome Measures: Anterior chamber inflammation and microhyphema, IOP, and corneal edema., Results: Anterior segment imaging demonstrated bilateral implant-iris apposition and implant-angle structures apposition. Specular microscopy demonstrated a significant reduction of the number of endothelial cells. VF test demonstrated a right superior arcuate scotoma and a normal left VF. Following surgical treatment IOP normalized and inflammation and microhyphema subsided. However, mild corneal edema persisted., Conclusion: Cosmetic iris implantation may lead to UGH syndrome and corneal decompensation. Therefore, its use should be reserved for patients with significant medical indications, and avoided in patients with intact natural iridies, until more short-term and long-term safety data are available.

Purpose: To describe clinical features of chronic anterior uveitis in children at presentation to a referral center (baseline); to identify relationships between demographic, medical, and ophthalmic factors at baseline; and to determine baseline factors that predict new complications and vision loss during follow-up., Design: Retrospective case series., Methods: Studied were involved eyes of all children (age < or =16 years at disease onset) with chronic anterior uveitis who were examined by 1 clinician from 1993 through 2006. Cross-sectional analyses compared baseline findings. Relationships between potential risk factors and incident adverse events (new complications, vision loss) were studied by Kaplan-Meier and Cox proportional hazards regression models., Results: There were 115 patients (200 eyes) who met inclusion criteria. Follow-up (n = 83 patients) ranged from 0.4 to 157.5 months (median, 23.5 months). There were numerous strong relationships between 8 defined complications at baseline in pairwise comparisons. Flare was the inflammatory sign most consistently associated with complications at baseline. Baseline factors that predicted new complications during follow-up included age < or =3 years, elevated cells, elevated flare, keratic precipitates, signs of intermediate uveitis, and papillitis (all P < .043); factors that predicted vision loss included male gender, increased flare, signs of intermediate uveitis, papillitis, and baseline complications (all P < .015). Not related to new complications were presence of juvenile idiopathic uveitis and immunomodulatory therapy., Conclusion: Chronic anterior uveitis in children is associated with various vision-threatening complications that occur in combinations. Complications develop early in the disease course. Patients with more severe disease at presentation are at increased risk of additional adverse events.

Purpose: To evaluate the anterior chamber (AC) inflammatory reaction by anterior segment high-speed optical coherence tomography (OCT)., Design: A prospective, nonrandomized, observational case series., Methods: Sixty-two eyes of 45 patients were studied for AC reaction clinically and by anterior segment OCT. Hyperreflective spots suggesting the presence of cells in the AC from the OCT images were counted manually and by a custom made automated software using MATLAB (Mathworks, Natick, Massachusetts, USA) and correlated with clinical grading of AC cells using Standardization of Uveitis Nomenclature criteria., Results: In manual method, mean hyperreflective spots were 3 +/- 1.8 in grade 1, 12 +/- 3.5 in grade 2, 33.8 +/- 10.2 in grade 3, and 61.4 +/- 9.6 in grade 4. Automated method showed mean 3 +/- 1.9 hyperreflective spots in grade 1, 12.4 +/- 3.6 in grade 2, 33.2 +/- 9.6 in grade 3, and 74.8 +/- 17 in grade 4. Significant difference seen in mean values between the manual and automated method in grade 4 (P = .009). AC cells were detected in 12 (19.3%) eyes with corneal edema with central corneal thickness ranging from 702 to 1020 microns (mean, 843 +/- 109 microns). Out of 62 eyes, grade 4 aqueous flare was detected by OCT imaging in 7 eyes and clinically in 5 eyes., Conclusion: Anterior segment OCT can be used as an imaging modality in detecting AC inflammatory reaction in uveitis and also in eyes with decreased corneal clarity and compromised AC visualization attributable to corneal edema. Automated method is sensitive in higher grades of uveitis.

Purpose: To compare the characteristics of cytomegalovirus (CMV)-positive and negative eyes with presumed Posner-Schlossman syndrome (PSS) and Fuchs heterochromic iridocyclitis (FHI)., Design: Retrospective interventional case series., Methods: One hundred and three eyes of 102 patients with presumed PSS or FHI, seen at the Singapore National Eye Centre, underwent aqueous analysis for CMV by polymerase chain reaction (PCR). Their records were reviewed for clinical features and human immunodeficiency virus (HIV) status of the CMV-positive patients. The main outcome measures were age, gender, maximum intraocular pressure, endothelial cell count, endothelial changes, PCR results, and presence of uveitic cataract and/or glaucoma., Results: Sixty-seven eyes with presumed PSS were tapped, of which 35 (52.2%) were CMV-positive. There were 36 eyes of 35 patients with presumed FHI, of which 15 (41.7%) were CMV-positive. All the CMV-positive patients were HIV negative. Nodular endothelial lesions were seen in 18 eyes (36.0%) with CMV infection, and reticulate deposits were seen in all the presumed FHI eyes. CMV-positive and CMV-negative PSS eyes were clinically similar. Older age at diagnosis, male gender, and nodular endothelial lesions were significantly associated with CMV infection in presumed FHI eyes (age: odds ratio [OR], 1.1; 95% confidence interval [CI], 1.0 to 1.2; P = .01; male gender: OR, 9.4; 95% CI, 1.0 to 88.6; P = .049; nodular endothelial lesions: OR, 13.9; 95% CI, 1.5 to 132.7; P = .02)., Conclusions: There are no clinically detectable differences between CMV-positive and negative presumed PSS eyes. CMV-positive presumed FHI patients are more likely to be male, older at diagnosis or have nodular endothelial lesions.

Purpose: To investigate the clinical profile of patients with chronic anterior uveitis and intraocular analyses positive for intraocular Rubella virus infection and assess eventual similarities to Fuchs heterochromic uveitis (FHU)., Design: Retrospective case-control study., Methods: Clinical records of 30 patients with anterior uveitis positive for intraocular antibody production against Rubella virus by Goldmann-Witmer coefficient determination and/or polymerase chain reaction were reviewed and compared with clinical records of 13 patients with chronic anterior uveitis of undetermined origin. Multiple variables were assessed and patient records were evaluated at onset and at one year after their first visit to the University Medical Center Utrecht., Results: Patients with Rubella virus-associated uveitis were younger at time of initial ophthalmologic presentation (P = .014). Rubella virus-positive patients presented more frequently with unilateral ocular disease (P < .001), keratic precipitates (KPs; P = .014), iris atrophy and/or heterochromia (P = .051), associated vitreous opacities (P = .024), and cataract (P = .004). Also, the combination of KPs, absence of posterior synechiae, cataract, and vitreous opacities occurred more often in the Rubella virus-positive group (P = .026) and the presence of three or four of these criteria occurred more frequently in the Rubella virus-positive group (P = .004)., Conclusions: Rubella virus causes a distinct clinical spectrum of ocular symptoms similar to the FHU syndrome, which suggests that Rubella virus might be involved in the pathogenesis of FHU.

Purpose: To estimate the incidences of ocular complications and vision loss in patients with juvenile idiopathic arthritis (JIA)-associated uveitis, to describe risk factors for vision loss, and to describe the association between therapy and complications and vision loss., Design: Retrospective cohort study., Methods: setting: Single-center, academic practice. study population: A total of 75 patients with JIA-associated uveitis evaluated between July 1984 and August 2005. procedures: Clinical data on these patients were analyzed. outcome measures: Occurrence of ocular complications and visions of 20/50 or worse and 20/200 or worse., Results: Over a median follow-up of three years, the incidence of any ocular complication was 0.33/eye-year (EY). Rates of vision loss to 20/50 or worse and 20/200 or worse were 0.10/EY and 0.08/EY, respectively. Risk factors at presentation for incident vision loss included presence of posterior synechiae, anterior chamber flare > or = 1+, and abnormal intraocular pressure (IOP). During follow-up, ocular inflammation > or = 0.5+ cells was associated with an increased risk of visual impairment (relative risk [RR] = 2.02, P = .006) and of blindness (RR = 2.99, P = .03). Immunosuppressive drug therapy reduced the risk of hypotony by 74% (P = .002), epiretinal membrane formation by 86% (P = .05), and blindness in the better eye by 60% (P = .04)., Conclusions: Incident vision loss and complications were common. Presence of posterior synechiae, anterior chamber flare > or = 1+, and abnormal IOP at presentation were associated with vision loss during follow-up. Use of immunosuppressive drugs reduced the risk of some ocular complications and of blindness in the better-seeing eye.

Purpose: To describe the frequencies of and risk factors for ocular complications and poor visual acuity at presentation in a cohort of patients with juvenile idiopathic arthritis (JIA)-associated uveitis., Design: Cross-sectional study., Methods: setting: Single-center, academic practice. study population: Seventy-five patients with JIA-associated uveitis were evaluated between July 1984 and August 2005. observation procedures: Data on patients diagnosed with JIA-associated uveitis were entered retrospectively into a database and analyzed. outcome measures: Visual acuity of 20/50 or worse or 20/200 or worse, and presence of ocular complications (including cataract, posterior synechiae, band keratopathy, elevated intraocular pressure, hypotony, macular edema, and epiretinal membrane) at presentation., Results: At presentation, ocular complications were seen in 67% of eyes affected by JIA-associated uveitis. Presence of > or =1+ anterior chamber flare, a positive antinuclear antibody (ANA), and a shorter duration between the diagnosis of arthritis and uveitis were significantly associated with the presence of ocular complication. The frequencies of 20/50 or worse and of 20/200 or worse visual acuities at presentation in affected eyes were 36% and 24%, respectively. The presence of > or =1+ anterior chamber flare and a history of intraocular surgery before presentation were significantly associated with 20/50 or worse and 20/200 or worse vision. Presence of posterior synechiae also was associated with 20/200 or worse vision at presentation. The main causes of poor vision at presentation for affected eyes and better-seeing eyes were cataract, band keratopathy within the visual axis, and glaucoma., Conclusions: Ocular complications and poor vision at presentation were common in our patients with JIA-related uveitis.

Purpose: To describe a case of uveitis that is associated with Borrelia hermsii relapsing fever., Design: Interventional case report., Methods: A 12-year-old boy with two weeks of relapsing fevers 10 days after camping in remote eastern Oregon was examined. Borrelia hermsii immunoglobulin M and G levels were markedly elevated. Intravenous ceftriaxone, followed by four weeks of oral cephuroxime was administered, but the patient developed unilateral floaters and blurred vision in association with anterior and intermediate uveitis., Results: Doxycycline was administered for presumed residual infection. Four weeks later, the visual acuity had improved. The anterior chamber was quiet, and topical corticosteroid was tapered successfully., Conclusion: Although rare, Borrelia hermsii should be included in the list of spirochetal diseases that are associated with uveitis.

Purpose: To describe a case of Langerhans cell histiocytosis (LCH) that involved the anterior uveal tract., Design: Interventional case report., Methods: A retrospective review was conducted on a patient with iris nodules and anterior uveitis in the setting of LCH. Visual acuity and clinical findings that were noted on slit lamp biomicroscopy were extracted., Results: An 18-year-old male patient with unilateral anterior segment inflammation and iris nodules experienced visual improvement from 20/200 to 20/25 after treatment with a 5-day course of topical corticosteroids. Regression of the iris nodules and anterior segment inflammation was also noted. Bone marrow aspirate confirmed recurrent, active LCH., Conclusion: The clinician should include LCH in the differential diagnosis when faced with anterior segment inflammation in conjunction with iris nodules. Additionally, LCH can be treated successfully with topical corticosteroid therapy.

Purpose: To describe the clinical characteristics and course of optic disk edema (ODE) associated with isolated sudden-onset anterior uveitis., Design: Interventional, noncomparative retrospective case series., Methods: Review of patients with sudden-onset anterior uveitis and concomitant ODE seen in an academic uveitis referral center or a general ophthalmology clinic between August 2001 and November 2002., Results: A total of eleven eyes of seven patients were included. Optic nerve function was intact in all. Cranial magnetic resonance imaging was normal in four of five and revealed an abnormality not felt to be responsible for the ODE in one. Resolution of anterior uveitis occurred over a mean of 36 days while ODE resolution lagged by a mean of 20 days., Conclusion: Resolution of ODE trailed that of the anterior uveitis by up to 6 weeks. No abnormalities responsible for ODE were found on cranial imaging.

Purpose: To report acute unilateral hypopyon uveitis as an initial presenting feature of relapsing acute lymphoblastic leukemia (ALL) in an adult patient., Design: Observational case report., Methods: Anterior chamber paracentesis was performed in a 56-year-old male presenting with treatment-resistant unilateral hypopyon while in the remission phase of ALL., Results: Examination of the aqueous humor aspirate revealed presence of malignant cells compatible with the previous bone marrow biopsy and subsequent spinal tap results., Conclusions: Atypical hypopyon uveitis can be an indication of relapsing ALL, even in adults. Prompt anterior chamber aspiration is required for the correct diagnosis and subsequent treatment.

Purpose: Anterior chamber involvement is unusual in patients with acute lymphoblastic leukemia (ALL) and has never been described in the setting of Ph+ (Philadelphia chromosome-positive) ALL. Moreover, there have been no reports of this complication as a primary relapse in a patient treated with imatinib., Design: Interventional case report., Methods: A 55-year-old woman with Ph+ ALL in complete remission with imatinib and presenting unilateral anterior uveitis at initial examination was clinically evaluated. Hematologic and ocular studies were performed: blood films, bone marrow smears, and anterior chamber paracentesis with aqueous fluid cytology., Results: Although there was no evidence of leukemia in the blood or bone marrow samples, aqueous fluid cytology identified Ph+ positive lymphoblastic leukemic cells., Conclusions: The patient had developed anterior chamber infiltration without hematological relapse while treated with imatinib. In our opinion, paracentesis should be performed without delay when uveitis develops in ALL, regardless of systemic relapse.

Purpose: To report a case of an occult, metallic, anterior-chamber intraocular foreign body after uneventful phacoemulsification that was masquerading as chronic recalcitrant postoperative inflammation., Design: Interventional case report., Methods: A 73-year-old patient was referred to us for recalcitrant anterior-chamber inflammation after uneventful phacoemulsification; the patient presented with visual disturbances, anterior-chamber inflammation, and macular epiretinal membrane with concomitant cystoid macular edema., Results: After meticulous evaluations and repeated clinical examinations, a metallic intraocular foreign body was discovered on the iris, which was surgically removed and analyzed. Chemical analysis revealed copper, aluminum, and zinc. Pars plana vitrectomy with epiretinal membrane and indocyanine-green-assisted internal limiting membrane peeling followed, with subsequent improvement of visual acuity., Conclusions: Intraocular foreign bodies should always be considered in the differential diagnosis of recalcitrant inflammation post-phacoemulsification. However, in the absence of intraocular inflammation, surgical removal of such particles is questionable.

Purpose: To describe the clinical characteristics of uveitis occurring in patients with psoriasis and to compare them with patients with idiopathic anterior uveitis and HLA-B27-associated anterior uveitis., Design: Case-control study., Methods: The charts of 36 patients with a diagnosis of uveitis and psoriasis visiting the Immunology and Uveitis Service at the Massachusetts Eye and Ear Infirmary between January 2000 and December 2001 were reviewed. Clinical characteristics, therapy, and outcomes of uveitis were compared with 30 randomly selected patients with either idiopathic anterior uveitis or with HLA-B27-associated anterior uveitis., Results: The mean age at presentation for uveitis was significantly higher in patients with psoriasis compared with nonpsoriatic groups (44.6 years in HLA-B27-psoriatic patients vs 35.9 years in HLA-B27- nonpsoriatic patients, P = .034; 52.7 years in HLA-B27+ psoriatic vs 35.7 years in HLA-B27+ nonpsoriatic patients, P = .001). Uveitis in patients with psoriasis also tended to be bilateral (62%) and prolonged (11.2 weeks). HLA-B27+ patients with psoriasis required supplemental therapy with oral nonsteroidal anti-inflammatory drugs (95% confidence interval [CI] 1.41 to 5.36, P = .003) for anterior uveitis more often than did the HLA-B27+ nonpsoriatic group. Patients with psoriasis and uveitis who were HLA-B27+ required oral NSAIDs (95% CI 1.51 to 9.79, P = .001) for anterior uveitis more often than did HLA-B27- psoriatic patients., Conclusion: Uveitis in patients with psoriasis may have distinguishing clinical features. Further epidemiologic studies are required to determine the strength of association between psoriasis without arthritis but with uveitis.

Purpose: To report the closure of a macular hole after a peribulbar corticosteroid injection in a patient with anterior uveitis associated with human leukocyte antigen B*27., Design: Interventional case report.The patient was evaluated by comprehensive ophthalmic check-up and optical coherence tomography., Results: A healthy 50-year-old woman presented to our service with active human leukocyte antigen B*27-associated anterior uveitis, macular hole in the right eye and macular edema in the left eye. Visual acuity was 20/200 in both eyes. Peribulbar injections of triamcinolone acetonide were administered in both eyes. Three months later, visual acuity improved to 20/50 in both eyes, and the uveitis was in complete remission. Optical coherence tomography verified that the macular hole in the right eye was closed., Conclusions: Inflammation and macular edema might be contributing factors in macular hole formation in patients with uveitis. This case indicates that treatment of inflammation and macular edema might lead to macular hole closure in similar cases.

Purpose: We describe important characteristics of the chronic arthritides of childhood associated with anterior uveitis., Design: Literature review and commentary based on the investigators' experiences., Method: A review of pertinent publications provides a background of current understanding of those forms of childhood arthritis that are of interest to ophthalmologists caring for children. Recommendations regarding screening for asymptomatic anterior uveitis in this patient group are reviewed, and current advances in therapy are noted. A new classification of childhood arthritis is compared with existing classifications., Results: Uveitis complicates several forms of juvenile idiopathic arthritis (oligoarthritis, rheumatoid factor-negative polyarthritis, psoriatic arthritis, and enthesitis-related arthritis). Uveitis is a complication in up to 30% of children with chronic arthritis, particularly in those with oligoarticular disease., Conclusions: Visual prognosis is guarded for children with chronic arthritis and uveitis. Surveillance and early therapy are important factors for ensuring a good outcome.

Purpose: Pediatric uveitis is rare and has been reported to cause increased rates of visual loss compared with adult patients. The reasons for this are unclear. Only one study has been population-based, so the effect of referral bias is not known. We examined the pattern of disease in primary and referral centers to establish the unique characteristics of uveitis in children., Design: Case control study., Methods: Retrospective, multicenter, observational study of uveitis starting before the age of 20 years. Two hundred forty-nine patients were recruited from three primary and two referral ophthalmic units. Age-related differences in types of uveitis and systemic disease between hospitals were characterized, as were associations with visual loss., Results: The incidence of uveitis in district hospitals at less than 16 years of age was 4.9/100,000: the most frequent diagnosis was idiopathic uveitis (78%). In referral cohorts the most frequent diagnosis was juvenile idiopathic arthritis-associated uveitis (67%). Other systemic diseases were rare. The most frequent type of uveitis at 0 to 7 years of age was chronic anterior uveitis, posterior uveitis in 8- to 15-year-olds, and acute anterior uveitis in 16- to 19-year-olds. Visual loss (any eye < 6/12) occurred in 17% and was not associated with age, sex, or hospital cohort. It was most frequent in posterior uveitis (25%). Treatment variables were independent predictors of visual loss: systemic treatment 2.2 (1.1- 4.6), surgical intervention 8.2 (3.8-17.6)., Conclusions: Idiopathic uveitis was three times more common in district hospitals. Visual loss was similar to adult uveitis in this study. The increased frequency of severe chronic anterior uveitis in children aged 0 to 7 years and posterior uveitis in older children aged 8 to 15 years accounts for the rate of visual loss seen in previous studies.

Purpose: To report a case of herpes zoster virus sclerokeratitis with anterior uveitis following vaccination with live attenuated varicella vaccine (Oka strain)., Design: Case report., Methods: The case records of the patient were reviewed retrospectively. Pertinent literature citations were identified using MEDLINE., Results: A 9-year-old boy presented with herpes zoster ophthalmicus 3 years following vaccination with live attenuated varicella vaccine (Oka strain). Examination of the affected eye revealed a moderate follicular response on the palpebral conjunctiva, decreased corneal sensation, mildly elevated intraocular pressure, diffuse anterior scleritis with marginal keratitis, and a moderately severe anterior uveitis. Amplified DNA from fluid taken from the base of a cutaneous vesicle produced wild-type varicella zoster virus (VZV) DNA, not Oka strain., Conclusions: Herpes zoster virus infection needs to be considered in all patients who present with scleritis, keratitis, or anterior uveitis, regardless of their varicella vaccination status., (Copyright 2003 by Elsevier Science Inc.)

Purpose: To evaluate ocular features in patients presenting with inflammation in the presence of anticardiolipin antibodies., Methods: A descriptive study of 13 patients presenting with idiopathic ocular inflammation involving anterior and posterior segment was performed. Patients were followed for a mean follow-up of 22 months (range, 1 to 125). A comprehensive report of ocular involvement, including visual symptoms, visual acuity, clinical characteristics, funduscopic and fluorangiographic features, was reported. Systemic associated symptoms were analyzed. Laboratory investigations included anticardiolipin antibody titers and isotypes, presence of other autoantibodies, and markers of immune system activation., Results: The most common ocular symptom at presentation was blurred vision (eight patients) followed by redness and pain(three patients) and visual loss(two patients). Anterior segment abnormalities, including iritis (eight patients) scleritis (two patients) and filamentary keratitis (one patient), were present in 76% of patients, whereas the most represented feature of posterior involvement was retinal vasculitis (60%) followed by vitritis (38%), retinal detachment (15%), posterior scleritis (7%), and central retinal artery occlusion (7%). All patients had abnormal titers of anticardiolipin antibodies, predominantly IgG isotype; six had markers of immune system activation., Conclusions: Although posterior pole disease is more commonly associated with anticardiolipin antibodies, the anterior segment can also be involved with a wide spectrum of features. Scleritis has never been previously described as associated with anticardiolipin antibodies. Systemic symptoms are frequently present in association with ocular disease.