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To describe a case of T-cell prolymphocytic leukemia (T-PLL) masquerading as viral retinal necrosis (VRN). A 75-year-old-man with a history of T-PLL in complete remission complained of an acute vision loss in his right and only eye. Ophthalmic examination demonstrated the presence of anterior chamber cells, mild vitritis, and peripheral retinal whitening with intraretinal hemorrhages evocative of VRN. While the anterior chamber tap came back negative for HSV, VZV, and CMV, cytology performed on the aqueous humor described the presence of leukemic cells. T-PLL can rarely masquerade as a viral retinal necrosis. Diagnostic work-up should therefore always rule out the infectious causes of retinitis. Anterior chamber tap can sometimes prove useful in the diagnosis of T-PLL even in the absence of a hypopyon, avoiding the need for vitrectomy. [ABSTRACT FROM AUTHOR]

Abstract: Background: The amount of specific antiviral IgG in aqueous humour (AH) provides a major contribution to the diagnosis of herpesvirus uveitis. Ocular antibody production is often evaluated by comparing levels of specific and total IgG in serum and AH. The small volume of AH is a major limit for diagnosis. Objectives: To simplify the measure of ocular antibody production, we tested the quotient of serum/AH ratios of specific and control antiviral IgG, using automated quantitative serology methods on minimal volumes of AH, in confirmed and suspected herpesvirus uveitis. Study design: Serum and AH samples from herpesvirus PCR-positive uveitis patients, and from PCR-negative cases who were highly suspected to have viral uveitis were retrospectively analysed for ocular production of specific antiviral IgG using 40μl of AH, and quantitative Enzygnost ELISA-based methods. Cataract and Fuchs cyclitis cases were used as controls. Results: Ocular production of specific antiviral IgG was demonstrated in 32 (51.6%) of 62 herpesvirus PCR-positive uveitis cases, in none of 42 controls, and in 21 (55.2%) of 38 PCR-negative cases clinically suspected to have herpesvirus uveitis. The test had absolute specificity, and its sensitivity depended on the virus, pathology and timing of sampling. Conclusion: Ocular antibody production can be measured by simple quantitative ELISA-based methods on serum and minimal volumes of AH. This specific and sensitive test, implemented in the routine virology laboratory should help the diagnosis and specific antiviral therapy management of herpesvirus uveitis. [Copyright &y& Elsevier]

Diagnosis of PIOL can be challenging. It requires a high degree of clinical suspicion and differential diagnosis includes infectious and non-infectious etiologies particularly the common masquaraders sarcoidosis, tuberculosis, viral retinitis and syphilis. The definitive diagnosis depends on demonstration of malignant lymphoma cells in ocular specimens or CSF. Ocular specimen could include vitreous, aqueous or chorioretinal biopsy. Ocular pathologist should be consulted prior to the diagnostic procedure to help handle and process the specimen appropriately. In addition to cytology, flow cytometry, immunohistochemistry, molecular analysis and cytokines may be used as adjuncts in facilitating the diagnosis. [ABSTRACT FROM AUTHOR]

Purpose: To report the cases of four patients with hepatitis C virus infection who experienced clinical features that are virtually identical to Vogt-Koyanagi-Harada disease (VKH).Design: Retrospective observational case series.Methods: Medical records of patients who were referred between January and December 2003 were reviewed for diagnosis and management of VKH and who also had chronic hepatitis C virus (HCV) infection.Results: Four white patients had the clinical features of VKH. Three of the patients experienced intraocular inflammation while they were being treated for HCV infection with pegylated interferon alpha 2b and ribavirin. The intraocular inflammation responded to systemic corticosteroid treatment and to discontinuation of antiviral agents.Conclusion: Although the number of patients who were studied is limited, there appears to be an association between HCV infection that was treated with pegylated interferon alpha 2b and the development of VKH-like disease. Further studies are required to confirm such an association. [ABSTRACT FROM AUTHOR]

Purpose Diagnosis of uveitis is difficult. Etiologic investigations should take into account the epidemiology of uveitis and should focus on the most severe forms of the disease and those which can be treated. This study was undertaken to establish recommendations for the diagnosis of uveitis. Methods Recommendations were developed by a multidisciplinary panel of 14 experts, including internists, ophthalmologists, and rheumatologists, and are based on a review of the literature and the results of the ULISSE study, which was the first prospective study to assess the efficacy of a standardized strategy for the etiologic diagnosis of uveitis. The following groups of patients are not included in these recommendations: children, immunocompromised patients, patients with severe retinal vasculitis, and those with specific eye diseases diagnosed by ophthalmologic examination only. Results Diagnosis should be guided by the medical history of the patient and physical examination. Serologic screening for syphilis is appropriate in all forms of uveitis. If uveitis is not diagnosed at this stage, investigations oriented by the anatomic characteristics of uveitis are proposed. These consist of assays for HLA-B27 (in unilateral acute anterior non-granulomatous uveitis), serum angiotensin-converting enzyme, interferon-gamma release, chest computed tomography (chronic uveitis), cerebral magnetic resonance imaging and anterior chamber tap with interleukin-10 analysis (intermediate or posterior uveitis in patients > 40 years-old). Other investigations prescribed in the absence of orientation are usually unhelpful. Conclusions A strategy is proposed for the etiologic diagnosis of uveitis. The benefit of more invasive investigations remains to be determined. [ABSTRACT FROM AUTHOR]

The article evaluates the effectiveness of fluorescein angiography (FA) in diagnosing and managing uveitis. The findings provide information on the changes in different structures and extent of uveitis but does not allow analysis of the choriocapillaris and the choroid. The results also highlight the role of other imaging techniques such as indocyanine green angiography and optical coherence tomography (OCT).

Basic science and clinical investigations in cancer research have contributed to our understanding of the genetic causes of various neoplasms and discovery of novel therapeutic interventions to fight malignancies such as lymphoma. During this exciting time, we have witnessed the advent of new technologies to further characterize primary intraocular lymphoma (PIOL), or retinal lymphoma, which is selected as the first “Disease of the Year” by Ocular Immunology and Inflammation. Different comprehensive aspects of PIOL, including epidemiology, clinical manifestations, diagnosis, pathophysiology, therapy, and animal models are discussed. The future of PIOL holds an opportunity to really understand the unique cytologic, histopathologic, physiological and immunologic features, as well as the genotypic traits (gene expression, interaction, polymorphism, epigenetics, etc.) and epidemiology. This information will empower us to truly make a difference in patients’ managements with this devastating disease. While most of this technology already exists, much work still needs to be done to make translational therapy a reality for PIOL patients in the future. [ABSTRACT FROM AUTHOR]

Objective: To report the clinical features and treatment outcomes in immunocompetent patients with anterior segment inflammation (ASI) related to human cytomegalovirus (HCMV) depending on their ethnic origin.Material and Methods: Multicenter retrospective study of 38 patients with at least one test, either HCMV-positive PCR or GWc.Results: Features of Posner-Schlossman syndrome were observed in 50% of the eyes, Fuchs heterochromic iridocyclitis in 13% of the eyes, chronic nonspecific anterior uveitis in 21% of the eyes, and corneal endotheliitis in 18% of the eyes. PCR and GWc were positive for HCMV in 50% and 96.2% of the eyes, respectively. Glaucoma was diagnosed in 50% of eyes. Treatment was oral valganciclovir in about half of the patients. Other treatments were intravenous ganciclovir and/or ganciclovir topical ointment and/or intravitreal ganciclovir.Conclusions: No obvious association of specific clinical features with individual ethnicity could be identified. We found a high rate of glaucoma in all ethnic groups. There was a delay in diagnosis and specific treatment of HCMV in most patients. [ABSTRACT FROM AUTHOR]

Purpose ULISSE is the only study that prospectively assessed the efficiency of a standardized strategy, compared to an open strategy for the etiologic diagnosis of uveitis. Our aim was to evaluate the diagnostic yield of the tests prescribed in the ULISSE study to clarify their relevance. Methods ULISSE is a non-inferiority, prospective, multicenter and cluster randomized study. The standardized strategy is a two-steps strategy: in the first step, common standard tests were performed, and in the second step, tests were guided by the clinical and anatomic type of uveitis. We reported the relevance of the diagnostic tests used in the standardized strategy, as well as the profitability of the tests that were prescribed to more than twenty patients in each group. Based on diagnostic criteria, either an ophthalmologist, or an internist, established the profitability of a test by considering whether the test lead to a diagnosis or not. Results Among the 676 patients included (standardized 303; open 373), a diagnosis was made for 152 (50.4%) in the standardized group and 203 (54.4%) in the open group. The most common entities were HLA-B27 associated uveitis (22%), spondyloarthritis (11%), sarcoidosis (18%), tuberculosis (10.7%) and herpes virus infections (8.5%). Among the first step's systematic tests, tuberculin skin test was the most contributive investigation (17.1%), followed by chest X-ray (8.4%), C reactive protein and ESR (6.6% and 5.1%), complete blood count (2.2%) and VDRL (2.0%). The second step's most often contributive tests were: HLA B27 (56.3%), chest-CT (30.3%) and angiotensin converting enzyme (ACE) (16.5%). HLA B27 and ACE were significantly more contributive in the standardized group than in the open group. Immunological tests were never contributive. Among the free investigations, or among the investigations guided by clinical or paraclinical findings, the most often contributive tests were: Quantiferon® (24%), electrophoresis of serum protein (7.8%) and sacroiliac imagery (46.4%). Intracellular serologies (1.7%), serum calcium (2.1%) and hepatic tests (3.3%) were exceptionally contributive. Among the third intention tests, labial salivary gland biopsies were contributive in 17.9% of cases, but the profitability of other invasive investigations (anterior chamber tap, vitrectomy, bronchoscopy and lumbar puncture) or specialized imagery (18F-FDG PET, Brain MRI) could not be determined since these test were rarely performed. Conclusion Only a few diagnostic tests are useful for the etiological assessment of uveitis. They are often cheap, simple, more often guided by the clinical findings, and lead to an etiological diagnosis in most patients. On the other hand, some tests are never or exceptionally contributive, such as immunological tests or intracellular serologies. Further studies are required to evaluate the profitability of third intention imagery and invasive investigations. [ABSTRACT FROM AUTHOR]

Introduction: Chronic uveitis treated with immunosuppressive agents may have unfavorable outcomes due to delays in diagnosis. The aim of this study was to review initial data from clinical and ocular assessments and patient outcomes following specific treatments for atypical herpes virus ocular infections.Methods: The records of four consecutive patients with recurrent uveitis for at least five years treated in our department between 2001 and 2016 were retrospectively reviewed. They had atrophic retinal lesions resulting in an unfavorable outcome with lesion progression and vision loss under immunosuppressive treatment. Varicella zoster virus (1 case) and herpes simplex virus type 2 (3 cases) were detected in ocular samples.Results: Uveitis was granulomatous and bilateral (3 cases) or unilateral (1 case). Immunosuppressive treatments worsened the clinical situation whereas antiherpetic treatments improved the control of intraocular inflammation.Conclusion: This description should be added to the broad repertoire of clinical polymorphisms associated with herpes virus infections. Recognizing these cases should be useful due to their sensitivity to antiherpetic treatments. [ABSTRACT FROM AUTHOR]

Purpose: Few data are available regarding the optimal treatment of multiple sclerosis (MS)-related uveitis. The aim of this study was to describe clinical features of MS-associated uveitis and determine how MS treatment affects the course of uveitis.Methods: Retrospective, multicenter study. Patients were divided into two groups according to the use (group 2) or not (group 1) of immunomodulatory drugs. Characteristics of uveitis and treatment were reviewed.Results: A total of 68 eyes from 36 patients (17 in group 1 and 19 in group 2) were included. All patients were treated with topical and/or systemic steroids for uveitis. Uveitis occurred 1-17 years prior to neurologic symptoms in 78% of patients. Uveitis was more severe in group 2 (p<0.05), with a tendency toward a higher rate of chronic uveitis (p = 0.06).Conclusions: MS-related uveitis has often a favorable evolution. Patients on interferon-beta have more severe and chronic uveitis. As far as we are concerned, interferon-beta given on the sole indication of uveitis is not recommended. If steroid-sparing agent is required for intraocular inflammation, immunosuppressive drugs should be considered. [ABSTRACT FROM AUTHOR]

Purpose: To retrospectively assess the frequency of ocular relapse and the possibility of long-term remission in patients treated with interferon (IFN) for severe uveitis associated with Behçet disease.Methods: All patients were treated with an initial dosage of 3 million IU IFN three times a week. The main outcome measure was the number of relapses per person per year before, during, and after IFN treatment.Results: Of 36 patients (67 eyes), 31 (86.1%) responded to IFN. The mean follow-up was 8.19 years. Twenty-one out of 36 patients discontinued IFN and 76% of these have not relapsed within 5.05 years after discontinuation. The mean relapse per person per year decreased significantly from 1.39 to 0.0496 (p = 1.82×10-10) during the treatment period and remained at 0.057 relapses per person per year after IFN discontinuation.Conclusion: IFN efficiently decreases the relapse rate and seems to permit long-term remission even after discontinuation. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the changes of outer retinal tubulations (ORTs) as seen on spectral-domain optical coherence tomography (SD OCT) in eyes with neovascular age-related macular degeneration (AMD) where treatment was switched from intravitreal ranibizumab to intravitreal aflibercept. Methods: This was a prospective study of eyes diagnosed with neovascular AMD and previously treated with >6 intravitreal ranibizumab injections and switched to aflibercept, conducted at a single centre (Department of Ophthalmology at Pitié Salpetriere Hospital, Paris VI University) from January to July 2015. Before and after treatment was switched from ranibizumab to aflibercept, SD-OCT was used to evaluate the presence of ORTs. Additional assessments in this patient group included best-corrected visual acuity (BCVA), fluorescein angiography (FA), indocyanine green angiography (ICGA). Changes in pigment epithelium detachments (PED), presence of intraretinal cysts, and presence of subretinal fluid (SRF) were also noted. Results: Twenty-four eyes of 24 consecutive patients (15 female/nine male, mean age 70 years) diagnosed with neovascular AMD and previously treated with >6 intravitreal ranibizumab injections and switched to aflibercept were included in the analysis. After receiving aflibercept, patients were followed for a mean of 6.1 months. Prior to treatment switch, 97 % of eyes showed ORTs, while after treatment switch to aflibercept, at the end of the study period, 75 % had ORTs ( p = 0.219). Changes in BCVA (LogMAR) were not statistically significant (1.16 ± 0.44 to 1.18 ± 1.06, p = 0.12), however, a significant reduction in central macular thickness (CMT) (from 406 μm ± 112 to 263 μm ± 68, p = 0.001), PED (from 70.8 % to 41.7 % , p = 0.016), presence of intraretinal cysts (from 83.3 % to 33.3 %, p = 0.002) and SRF (from 91.7 % to 25 %, p = 0.001 ) were noted. Conclusion: After switching from ranibizumab treatment to aflibercept, ORTs remained present in 75 % of eyes, and significant reductions in CMT, PED, and SRF, and presence of intraretinal cysts were observed. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: Uveitis is the most severe complication of juvenile idiopathic arthritis (JIA). The diagnosis may be delayed, as visual symptoms are usually absent, which provides time for insidious complications. The course is chronic and long-term treatment is therefore required. Methods: We retrospectively reviewed the medical records of 75 children with uveitis who accounted for 10.5% of all patients with JIA seen at our pediatric rheumatology outpatient clinic between July 1997 and July 2007. Results: Uveitis occurred chiefly in patients with pauciarticular JIA. At last follow-up, in 69 patients in whom the files could be satisfactorily reviewed, only the anterior compartment was involved in 59.4% and both the anterior and the posterior compartments in the remainder. Mean age at the uveitis diagnosis was 4.5 years. In 9 (13.0%) patients, uveitis antedated joint manifestations; in 17 (24.7%) both were diagnosed simultaneously; and in 43 (62.3%) arthritis antedated uveitis. In 42 (61%) patients, complications occurred (synechiae, papillary block, cataract, hyalitis, papilledema, glaucoma, macular edema, elevated intraocular pressure, vision loss, and hypotonia). Topical medications were used in all patients for at least 3 months. Severe ocular involvement required systemic glucocorticoid therapy in 29 (42.0%) patients. Among immunomodulating agents, methotrexate and cyclosporine were used in 41 patients and TNFα antagonists in 15 patients. Surgery was performed in 21 (30.4%) patients. Uveitis completely resolved in 12 (17.4%) patients, a relapsing course occurred in 14 (20.3%), and became chronic with relapses as soon as the topical treatment was decreased in 23 (33.3%). A severe course was observed in 21 (30.4%) patients of whom 3 became blind and 4 lost vision in one eye. Conclusion: Uveitis is a severe complication of JIA. Patients with JIA should receive routine ophthalmological follow-up at regular intervals, even is their joint disease is quiescent. [Copyright &y& Elsevier]

Purpose: To investigate long-term responsiveness of patients with severe uveitis associated with Behçet disease to interferon alpha2a (IFN-alpha2a).Design: Retrospective study.Methods: Thirty-two patients with sight-threatening uveitis associated with Behçet disease who relapsed despite corticosteroids and immunosuppressive agents were included. IFN-alpha2a was administered subcutaneously (three million units thrice a week). Ophthalmologic examination and fluorescein angiography associated with laboratory tests were performed at regular intervals. Main outcome measures were visual acuity and recurrence of uveitis attacks before, during, and after initiation of IFN-alpha2a therapy.Results: Control of inflammation was achieved in 28 of 32 patients (88%). Mean observation period of the 28 responder patients was 70.6 months (range, 30.3 to 129.2 months). Median visual acuity improved from 0.52 to 0.33 (logarithm of the minimum angle of resolution units; P = .005) two years after initiation of IFN-alpha2a therapy. The relapse rate decreased significantly during IFN-alpha2a treatment from 1.68 +/- 1.22 relapses/patient/year to 0.11 +/- 0.20 relapses/patient/year (P < .0001). IFN-alpha2a was discontinued in 19 of 28 patients (68%) after 32 months of treatment (range, 16 to 50 months). Mean follow-up after IFN-alpha2a discontinuation was 43 months (range, 11 to 84 months). After IFN-alpha2a discontinuation, the relapse rate increased from 0.08 +/- 0.21 relapse/person/year to 0.74 +/- 1.40 relapse/person/year (P = 0.04).Conclusions: IFN-alpha2a is efficient and safe for the long-term management of severe uveitis associated with Behçet disease. Meanwhile it seems to be a suspensive therapeutic strategy, even though long-term remission is possible in some patients. [ABSTRACT FROM AUTHOR]

Behçet's disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage. The aim of this review is to provide current data on how innovations in clinical evaluation, investigations and treatments were able to improve the prognosis of uveitis associated with BD. [ABSTRACT FROM AUTHOR]