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

Purpose: To investigate the effect of interferon alfa-2a on T regulatory (Treg) cells, T helper 17 (Th17) cells, and expression of Toll-like receptors (TLRs) in Behçet disease (BD) patients with uveitis., Methods: Twenty-seven patients who received interferon alfa-2a for active BD uveitis despite conventional immunomodulatory therapies and healthy controls were enrolled. Peripheral blood Treg and Th17 cell frequencies were determined by flow cytometry as gated cells for CD3+CD4+Foxp3+ and CD3+CD4+IL17A+, respectively. Th17 RAR-related orphan receptor (ROR)γt mRNA expression was verified by real-time PCR (RT-PCR). Treg and Th17 cell cytokines were detected by ELISA in the supernatant of short-term cell cultures. RT-PCR was used to assess expression of TLR-2, TLR-3, TLR-4, TLR-8, and TLR-9 using cDNA prepared from CD4+ T cells and monocytes., Results: Treg and Th17 cell frequencies and Th17 RORγt expression were significantly elevated, and IL-10 concentration in Treg cell supernatants was significantly lower in BD patients than in controls. Th17 IL-17, IL-6, IL-21, IL-22, IL-23, IFN-γ, and TNF-α concentrations were significantly higher and all TLR expressions were significantly elevated in patients. Interferon alfa-2a led to a significant reversal in Treg and Th17 cell frequencies, Th17 RORγt expression, Treg and Th17 cell cytokine production, and TLR expression by CD4+ T cells and monocytes., Conclusions: Despite a relative increase in Treg cells, impaired IL-10 production suggests that Treg dysfunction may play a role in induction of BD uveitis. Favorable effects of interferon alfa-2a may be associated with recovery of Treg cell function, suppression of Th17 cells, and reduced expression of TLRs on CD4+ T cells and monocytes.

Purpose: To screen for psychological disorders in patients with active uveitis., Methods: Patients were screened for depression (BDI-II), state anxiety (STAI-I), VR-QOL (NEI-VFQ-25), and HR-QOL (SF-36). Association of depression and anxiety with sociodemographic and clinical parameters and with VR-QOL and HR-QOL were analyzed. Multivariate linear regression models were constructed for NEI-VFQ-25 and SF-36 subscales., Results: Of 99 patients, 37.3% screened positive for depression and 52.5% for anxiety. Depressed patients had lower visual acuity in the better seeing eye (p = 0.013) and more frequently panuveitis (p = 0.018). Anxious patients were younger (p = 0.009), had earlier onset of uveitis (p = 0.015), and had more frequently panuveitis (p = 0.016). Bivariate comparisons showed significant associations between psychological disorders and VR-QOL and HR-QOL. Significant bivariate associations were mostly lost in multivariate analyses for anxiety, but were preserved for depression., Conclusions: A positive screening test for depression and anxiety is common in patients with uveitis. Low vision and panuveitis are associated with depression. Depression is associated with impairment of VR-QOL and HR-QOL.

Behçet disease is a chronic relapsing multisystem inflammatory disorder. Ocular involvement is characterized by a bilateral recurrent non-granulomatous panuveitis and retinal vasculitis. Posterior segment findings vary during the course of the disease, in parallel with the relapsing and remitting intraocular inflammation. Structural alterations occur with increased disease duration. Fluorescein angiography is the gold standard in revealing the extent and severity as well as the leaky and/or occlusive nature of retinal vasculitis. Multimodal imaging using color fundus photography, fluorescein angiography, and optical coherence tomography is essential in visualizing diagnostic features, detecting structural changes, and monitoring disease activity and response to treatment in patients with Behçet uveitis.

Optical coherence tomography (OCT) is a non-contact noninvasive technique that allows in vivo imaging of the retina, choroid, optic nerve head, retinal nerve fiber layer, and the anterior structures of the eye. It was introduced into clinical practice two decades ago. Advances in OCT technology have been achieved by searching ultra-high-resolution OCT, adaptive optics OCT, eye-tracking OCT, and changes in signal detection technique from time-domain (TD) to spectral-domain (SD) detection. Today, SD OCT has become a part of routine uveitis practice. Apart from its diagnostic value in uveitis, OCT has enabled objective assessment of treatment response and provided predictive value for visual recovery and prognosis of uveitic entities. It is the standard diagnostic technique in the detection, monitoring of treatment, and determination of prognosis in uveitic macular edema as well as other inflammatory macular pathologies, including epiretinal membrane formation, vitreomacular traction, foveal atrophy, and lamellar/full-thickness macular holes. OCT has also shed light on the pathophysiology of several posterior uveitic entities. SD OCT has enabled visualization of four lines in the sensory retina which represent the external limiting membrane, the photoreceptor inner and outer segment junction, the photoreceptor outer segment and the retina pigment epithelium junction, and the retina pigment epithelium-choriocapillaris complex. Thus, we have gained substantial information about the pathologic and structural changes in uveitic conditions with primary or secondary outer retinal involvement. SD OCT has also provided invaluable information on the inner retinal and the vitreoretinal interface changes in uveitic conditions. With the introduction of enhanced depth imaging, visualization of the choroid and choriocapillaries has become possible. Therefore, OCT has become an indispensible ancillary test in the diagnosis and management of inflammatory diseases involving the retina and/or the choroid. As OCT technology continues to develop further it will provide new insights into the retinal and choroidal structure and the pathogenesis of posterior uveitic entities.

Purpose: To compare Behçet uveitis patients who presented in the 1990s and 2000s., Methods: Records of 170 patients seen in 1990-1994 and 258 patients seen in 2000-2004 were reviewed. Presenting features were analyzed in the whole cohort. Uveitis attacks, vision, complications, and immunomodulatory treatment (IMT) were compared in patients with 3-year follow-up., Results: Posterior segment involvement was less common, visual acuity was better, and more patients received IMT before referral in the 2000s. Follow-up revealed no significant difference in number of uveitis attacks in the first 3 years. Less eyes lost useful vision, no patient became legally blind, and fewer severe ocular complications occurred in the 2000s. IMT was started within 3 months in more patients and interferon alfa-2a was used only in the latter study period., Conclusions: The findings suggest a milder disease at referral, lower rate of severe complications, and a better 3-year visual outcome in patients who presented in the early 2000s.

Objective: To investigate vision- and health-related quality of life in patients with Behçet disease., Methods: Fifty-one consecutive patients with Behçet uveitis were enrolled in the study from January 1 through June 30, 2008. The National Eye Institute Visual Functioning Questionnaire (NEI-VFQ-25) and the 36-Item Short Form Health Survey (SF-36) were administered. Sociodemographic and clinical data were also collected. Main outcome measures were comparison of the NEI-VFQ-25 and SF-36 subscale item scores among subgroups and multivariate analysis of the NEI-VFQ-25 and SF-36 subscale item scores., Results: Patients rated the general health subscale score of the NEI-VFQ-25 and all subscale item scores of the SF-36 lower than the NEI-VFQ-25 subscales related to vision. The NEI-VFQ-25 subscale item scores showed significant differences with respect to age, educational level, Behçet uveitis activity and severity, and visual acuity in the better and worse eyes. The SF-36 subscale item scores revealed significant differences according to sex, educational level, and the systemic treatment used. In the best model of linear regression, independent variables accounted for 57.0% of the variance in the NEI-VFQ-25 subscale item (color vision subscale; adjusted R² = 0.57, P < .001) and for only 23.0% of the variance in the SF-36 subscale item score (role limitation owing to emotional problems subscale; adjusted R² = 0.23, P = .004)., Conclusions: General health is more affected than visual functioning in patients with Behçet uveitis. Sociodemographic and clinical variables had a significant effect on vision- and health-related quality of life. Multivariate analysis of the NEI-VFQ-25 and SF-36 subscales revealed that each subscale item score is affected by additional factor(s) other than those analyzed here.

Purpose: To analyze the referral patterns and clinical features of uveitis in tertiary eye care centers in Turkey., Methods: Uveitis services of eight university clinics collected data on uveitis patients who presented during 2004. Data were collected on a form including a questionnaire on socio-demographic and clinical features which were documented at presentation., Results: A total of 761 patients (1187 eyes) with a mean age of 35.5 years were included in the study. Male to female ratio was 1.04:1. The majority of patients (89.6%) were from an urban population, 53% were referrals and 43% had had uveitis for more than one year. The most common type of uveitis was anterior uveitis (52.5%) followed by panuveitis (28.1%), posterior uveitis (12.7%) and intermediate uveitis (6.7%). In 56.8% of patients an etiological classification was established. The most common etiology was Behcet's disease (32.1%), followed by Fuchs' heterochromic iridocyclitis (5.1%) and ocular toxoplasmosis (4.7%). Among 48 pediatric patients uveitis associated with juvenile idiopathic arthritis and ocular toxoplasmosis both having equal frequencies (12.5%) were the most common diagnosis. These were followed by Behcet's disease with juvenile-onset (10.4%). At the time of presentation, 6.25% of the patients were legally blind (having visual acuity less than 0.1 in the better seeing eye)., Conclusion: Compared to the uveitis series reported from other countries, Behcet uveitis was the leading cause of uveitis in this series. Uveitis associated with juvenile idiopathic arthritis and ocular toxoplasmosis were the most common cause of uveitis in childhood. On the other hand, entities like acquired immunodeficiency syndrome-related uveitis, presumed ocular histoplasmosis and Birdshot retinochoroidopathy were rare.

Purpose: To evaluate the short-term safety and efficacy of intravitreal bevacizumab for the treatment of cystoid macular edema (CME) secondary to uveitis., Design: Retrospective, noncomparative, interventional case series., Participants: Thirteen patients undergoing treatment for recalcitrant uveitic macular edema at one referral center., Methods: Charts of patients who received one 2.5-mg intravitreal injection of bevacizumab in one eye were reviewed for clinical information including best-corrected Snellen visual acuity (VA), examination findings, optical coherence tomography (OCT) results, and fluorescein angiography results. Kaplan-Meier survival analysis was used to calculate probability success rates. The statistical significance of change in mean retinal thickness and VA was assessed using repeated-measures analysis of variance., Main Outcome Measures: Assessments of changes in best-corrected Snellen VA and OCT retinal thickness were made., Results: Six (46.15%) patients had a decrease in foveal thickness at the end of the follow-up, whereas 5 (38.4%) patients had an improvement of VA by > or =2 lines 84 days or more after the injection. Mean retinal thickness showed a significant decrease over the follow-up (P<0.02). The change in mean logarithm of the minimum angle of resolution VA over the follow-up was not significant (P>0.05). Survival analysis showed that the probability of any improvement in VA increased progressively starting at 6 weeks and reached 81% at 14 weeks. No significant ocular or systemic adverse effects were observed., Conclusions: These results suggest that a single intravitreal injection of bevacizumab is well tolerated and is associated with short-term improvement in VA and decreased OCT retinal thickness in a considerable proportion of patients with uveitic CME resistant to conventional therapy. Further evaluation of intravitreal bevacizumab for uveitic CME in controlled randomized studies is warranted.

Purpose: We aimed to describe the demographic and clinical features, ocular manifestations, complications, visual prognosis, and treatment in a large population of Turkish patients with Behçet uveitis. We also aimed to compare visual prognosis between male and female sex and between patients who presented before and after 1990., Design: Observational case series., Methods: A retrospective study of 880 consecutive patients (1,567 eyes) with Behçet uveitis seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, from 1980 to 1998. All patients met the classification criteria of the International Study Group for Behçet's Disease. Information on the patient's sex, age at onset of uveitis, ocular features, ocular complications, visual acuity, and systemic treatment was collected., Results: Five hundred ninety-nine patients (68%) were male and 281 (32%) were female. The mean age at onset of uveitis was 28.5 years in male and 30 years in female patients. Ocular involvement was bilateral in 78.1% and unilateral in 21.9% of the patients. Panuveitis was the most common form in both sexes. Fundus lesions as well as sight-threatening complications were more common in males. At the beginning of the follow-up, potential visual acuity was 0.1 or less in 30.9% of eyes in males and 24.2% of eyes in females. Kaplan-Meier survival analysis estimated the risks of losing useful vision (>0.1) at 5 and 10 years for males and females as 21% vs 10% and 30% vs 17%, respectively. Male patients who presented in the 1990s had a significantly lower risk of losing vision compared with male patients who presented in the 1980s., Conclusion: Behçet uveitis starts frequently around the end of the third decade and has a male predominance. The disease is more severe and the risk of losing useful vision is higher in males than in females. However, this risk has been significantly reduced in the 1990s.

Purpose: To report the efficacy and safety of interferon (IFN) α-2a in patients with cystoid macular edema (CME) associated with presumed ocular tuberculosis (TB).Methods: We reviewed the clinical records of 5 patients with presumed ocular TB who had been treated with IFN α-2a for recurrent CME during or after completion of anti-tubercular therapy. IFN α-2a was administered at an initial dose of 3 million IU per day and then tapered after the initial response. Treatment efficacy was assessed by central macular thickness (CMT) measurement using spectral-domain optical coherence tomography and visual acuity.Results: Three patients were men, and 2 were women. Patients were aged between 38 and 66 years. Mean CMT was 483 ± 178.6 μm at baseline, 302.3 ± 56 μm at 1 week, 312.3 ± 49.5 μm at 1 month, and 286.2 ± 31.9 μm at 3 months. Mean LogMAR visual acuity was 0.6 ± 0.4 at baseline, 0.4 ± 0.3 μm at 1 week, 0.3 ± 0.3 at 1 month, and 0.3 ± 0.3 at 3 months. The treatment was interrupted for 10 days because of neutropenia after 2 weeks in 1 patient and discontinued in another after 10 days because of intolerance. Total treatment duration was 3-24 months in the remaining 4 patients.Conclusion: The present small case series suggests that IFN α-2a may be an effective and safe therapeutic option for CME that is associated with presumed ocular TB. [ABSTRACT FROM AUTHOR]

Uveitis is one of the fields in ophthalmology where a tremendous evolution took place in the past 25 years. Not only did we gain access to more efficient, more targeted, and better tolerated therapies, but also in parallel precise and quantitative measurement methods developed allowing the clinician to evaluate these therapies and adjust therapeutic intervention with a high degree of precision. Objective and quantitative measurement of the global level of intraocular inflammation became possible for most inflammatory diseases with direct or spill-over anterior chamber inflammation, thanks to laser flare photometry. The amount of retinal inflammation could be quantified by using fluorescein angiography to score retinal angiographic signs. Indocyanine green angiography gave imaging insight into the hitherto inaccessible choroidal compartment, rendering possible the quantification of choroiditis by scoring indocyanine green angiographic signs. Optical coherence tomography has enabled measurement and objective monitoring of retinal and choroidal thickness. This multimodal quantitative appraisal of intraocular inflammation represents an exquisite security in monitoring uveitis. What is enigmatic, however, is the slow pace with which these improvements are integrated in some areas. What is even more difficult to understand is the fact that clinical trials to assess new therapeutic agents still mostly rely on subjective parameters such as clinical evaluation of vitreous haze as a main endpoint; whereas a whole array of precise, quantitative, and objective modalities are available for the design of clinical studies. The scope of this work was to review the quantitative investigations that improved the management of uveitis in the past 2-3 decades. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate agreement and estimate sensitivity and specificity of uveitis specialists' interpretation of ocular photographs in diagnosing Behçet uveitis. Methods: Fourteen Turkish uveitis specialists, masked to demographic and clinical features of patients, independently labeled ocular photographs (29 Behçet/30 other diagnoses) as 'Behçet uveitis' or 'non-Behçet.' Level of agreement was evaluated using kappa statistics. Photographs were categorized based on ocular signs captured and performance of observers. Results: Exact agreement with the correct diagnosis was 56-81%. Seven reviewers correctly labeled more than 70% of photographs. Interobserver agreement among those 7 reviewers revealed moderate ( κ = 0.41-0.60) or substantial ( κ = 0.61-0.80) agreement in 76% of pairs. Smooth layered hypopyon, superficial retinal infiltrate with retinal hemorrhages, and branch retinal vein occlusion with vitreous haze were correctly recognized as Behçet uveitis by majority of reviewers. Conclusions: There are ocular signs of Behçet disease that can be considered diagnostic even in the absence of any other clinical information. [ABSTRACT FROM AUTHOR]

Purpose: To establish evidence-based recommendations regarding the use of anti-Tumor Necrosis Factor alpha (TNF-α) agents for managing uveitis patients. Methods: Medline was searched via OVID (1950 - October Week 3, 2011) using a Cochrane highly sensitive search (phases 1 and 2). Additional literature searches were also conducted incuding the following databases: the Cochrane, LILACS and the TRIP Database. Results: A total of 54 studies met all of the inclusion criteria and were included in this review. A different level of recommendation and evidence is assigned to each anti-TNF-α agent. The overall rate of reported side effects with anti-TNF-α agents for the treatment of uvetis which required discontinuation of therapy was 2.2% (26/1147 patients). Conclusion: Based on the evidence gathered, infliximab and adalimumab seem to be effective in the management of immune-mediated uveitis. Further randomized studies evaluating the efficacy of these agents are warranted. It is the most common cause of inflammatory eye disease, with an estimated prevalence of 115 cases per 100,000 persons.1 Endogenous or associated with a systemic disease, noninfectious uveitis accounts for approximately 75% of total cases comprising of a heterogeneous group of inflammatory conditions responsible for about 10% of legal blindness in developed nations.1,2 Endogenous uveitides are thought to have an autoimmune component mediated by T lymphocytes specific to intraocular antigens that have failed to successfully pass basic processes designed to maintain self-tolerance. [ABSTRACT FROM AUTHOR]

Recent studies on the clinical importance of tuberculin skin tests (TST), interferon-gamma release assays (IGRAs), chest computed tomography (CT) and polymerase chain reaction have provided a new approach to diagnosing ocular tuberculosis (TBC). However, both TST and IGRAs cannot distinguish between latent TBC infection and active disease. Another corroborative test in the diagnosis of intraocular TBC is the evaluation of the lungs by either chest radiography or CT. Direct evidence for the diagnosis can be obtained by examination of smears and staining for acid-fast organisms, cultures of intraocular tissue/fluid for Mycobacterium tuberculosis, and nucleic acid amplification analysis. In recent years, guidelines have been suggested for the diagnosis of intraocular TBC. The current treatment of intraocular TBC consists of use of four drugs (isoniazid, rifampicin, ethambutol and pyrazinamide) taken for a long period of time (total 9-15 months). [ABSTRACT FROM AUTHOR]

The World Health Organization has declared tuberculosis (TB) to be a global emergency, as it remains the most common single cause of morbidity and mortality worldwide. TB is caused by the acid-fast bacillus Mycobacterium tuberculosis and primarily affects the lungs [pulmonary TB (PTB)]. It can also affect any other part of the body [extrapulmonary TB (EPTB)]. It is estimated that 1.4% of patients with PTB will eventually develop ocular disease; however, in the majority of cases of ocular TB, PTB may not be documented. Ocular TB infection is usually a result of hematogenous spread during PTB or EPTB. Symptomatic disease most commonly develops after reactivation of dormant foci in the ocular tissue rather than being the manifestation of the initial infection. Immune-mediated ocular TB can occur due tohypersensitivity to M. tuberculosis antigens from a distant focus (such as lungs), despite the absence of the bacterium in the eye. The most common clinical presentation of intraocular inflammation (uveitis) due to TB appears to be posterior uveitis, followed by anterior uveitis, panuveitis and intermediate uveitis. The absenceof uniform diagnostic criteria for intraocular TB has led to confusion regarding its diagnosis and management. Recent studies on the clinical importance of purified protein derivative (PPD) skin test, interferon-gamma release assays, chest computed tomography and polymerase chain reaction have provided a new approach to diagnosing ocular TB. This review series focuses on the clinical features, diagnostic techniques, diagnostic criteria, and treatment modalities in the light of recent literature. [ABSTRACT FROM AUTHOR]

Purpose: To assess the efficacy of intravenous immunoglobulin (IVIg) therapy in patients with severe uveitis otherwise unresponsive to conventional immunomodulatory agents. Methods: Data on five consecutive patients treated with IVIg and followed to the present time by one of the authors (CSF) were reviewed. All patients had severe and recalcitrant uveitis of diverse etiologies. Main outcome measures were control of intraocular inflammation, steroid-sparing effect, visual acuity, and side effects. Results: The duration of IVIg therapy was 3 to 36 months (mean, 16.8 months). Treatment was effective in controlling the intraocular inflammation in 3 of 5 patients. One of those patients required maintenance of systemic steroids at a dose of 10 mg per day. Visual acuity has stabilized or improved in these three patients. No immediate or long-term side effect was observed in any of the patients. Conclusions: Intravenous immunoglobulin therapy was an effective therapeutic modality in the treatment of three of five patients with severe uveitis that was unresponsive to conventional immunomodulatory agent(s). No adverse events were observed. [ABSTRACT FROM AUTHOR]

Purpose: To report a child who developed kerato-uveitis associated with mumps infection.Methods: A prospective study of a child who was observed at the Department of Ophthalmology, Marmara University, Faculty of Medicine, during spring 2003.Results: An 11-year-old male patient noticed decreased vision in his left eye. His right eye was completely normal. Visual acuity was perception of hand movements in the left eye. Ocular examination revealed ciliary injection and diffuse hyperemia of the conjunctiva, interstitial keratitis characterized by prominent stromal infiltrates and edema, folds in the Descemet's membrane, and microcystic epithelial edema in his left eye. The patient had been diagnosed as having mumps 10 days previously by a pediatrician. Complete recovery of the keratitis occurred on the 10th day of topical steroid treatment.Conclusions: Keratitis and/or iritis are rare complications of mumps. Corneal involvement is characterized by unilateral and painless interstitial keratitis that may cause a significant decrease in vision. Keratitis resolves with treatment and does not have any sequelae. [ABSTRACT FROM AUTHOR]