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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. [ABSTRACT FROM AUTHOR]

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. [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 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. [ABSTRACT FROM AUTHOR]

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. [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: Acute anterior uveitis (AAU), seronegative spondyloarthropathies such as ankylosing spondylitis and reactive arthritis form the group of “HLA-B27-associated diseases.” The aim of this study was to analyze the prevalence and levels of serum antibodies against gram negative bacteria in Turkish patients with AAU. Methods: Twenty-five patients each with previously diagnosed HLA-B27 positive and negative AAU and 25 age-and sex-matched healthy control subjects were included in the study. Serum IgM, IgG and IgA antibodies to Yersiniae enterocolitica, Campylobacter jejuni and Chlamydia trachomatis were measured using enzyme linked immunosorbent assay (ELISA). Categorical data were analyzed by chi-square test. Serum levels were analyzed using the Kruskal-Wallis test. Results: The prevalence of serum IgM, IgG and IgA antibodies did not differ significantly between the HLA-B27 positive AAU, HLA-B27 negative AAU and control groups with the exception of serum IgA antibodies against Yersiniae enterocolitica. IgA antibody against Yersiniae enterocolitica was found to be more frequently positive in the control group. Comparison of serum IgM, IgG and IgA antibody levels did not significantly differ between three groups. Conclusions: Serum antibody positivity against Yersiniae enterocolitica, Campylobacter jejuni and Chlamydia trachomatis is not frequent in the HLA-B27 positive and negative AAU patients. Serum levels of IgM, IgG, and IgA antibodies also did not show significant difference between three groups. No association between these microorganisms and the etiology of AAU was evident. [ABSTRACT FROM AUTHOR]

Purpose: Familial occurrence has been reported in approximately 8% of Turkish patients with Behçet's disease. Our aim was to compare the clinical course of ocular Behçet's disease among siblings.Methods: We retrospectively studied five pairs of siblings with ocular involvement of Behçet's disease. No other family member of these siblings had any symptom of Behçet's disease.Results: The siblings comprised three sister-brother pairs, one sister-sister pair, and one brother-brother pair. There was a 5-10-year difference between the age at onset of ocular disease among siblings. Three of the five pairs (two sister-brother, one brother-brother) had a dissimilar course of disease activity. Female siblings had an earlier age at onset and tended to have a worse prognosis than their brothers.Conclusions: It is generally acknowledged that male sex is associated with a worse prognosis. In this study, however, we observed that, among siblings with a dissimilar course, female patients were more severely affected than male patients. Poor ocular prognosis in a patient does not indicate aggressive treatment in his/her sibling. We believe that each sibling should be managed on an individual basis. [ABSTRACT FROM AUTHOR]

Purpose: The authors report a case of necrotizing scleritis associated with Wegener's granulomatosis (WG), which was treated with rituximab for relapsing disease. Method: Observational case report. Results: A 32-year-old male patient presented with necrotizing scleritis in his left eye. The patient was diagnosed as having limited WG. Cyclophosphamide was begun. Under maintenance treatment with azathioprine two relapses of scleritis occurred. Since a high cumulative dose of cyclophosphamide (22.5 g) was utilized initially, two intravenous infusions of rituximab 1 g was given. Complete resolution of scleritis occurred. Conclusions: Rituximab may be effective to induce remission in patients with scleritis due to WG. [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]

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]

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. [ABSTRACT FROM PUBLISHER]

Purpose: To describe 3 representative cases of autoimmune retinopathy (AIR). Methods: Clinical records of patients with a diagnosis of AIR were analyzed for demographic data, clinical findings, ancillary and laboratory tests, and treatment employed. Results: Three female patients diagnosed with AIR had bilateral reduction of electroretinogram amplitudes and elevation of visual field threshold within the central 30 degrees of the visual field that was disproportionately more severe than the clinical findings of retinal degeneration. The diagnoses were cancer-associated retinopathy, non-neoplastic AIR, and hereditary retinal dystrophy with secondary inflammation. Optic nerve involvement was also present in all cases. The patient with non-neoplastic AIR was successfully treated with systemic corticosteroids and immunomodulatory agents. Conclusion: High index of suspicion is essential for an early diagnosis of AIR. Visual function and electrophysiological tests should be included in the initial workup of patients who present with suggestive clinical signs and symptoms of AIR. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]