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International audience; PurposeTo describe the application of OCT-A in various posterior uveitis disorders in our experience and to compare it with the available literature.MethodsEighteen eyes with the diagnoses of multifocal choroiditis (MFC), multifocal placoid pigment epitheliopathy (APMPPE), multiple evanescent white dot syndrome (MEWDS), tuberculous serpiginous-like choroiditis (SLC), serpiginous choroiditis (SC), and birdshot chorioretinopathy (BSCR) were studied.ResultsWe found flow void of the choriocapillaris in patients with APMPPE, SC, MFC, BSCR, and in SLC. In contrast, perfusion of the choriocapillaris seemed normal in patients with MEWDS.ConclusionsWe confirmed that OCT-A contributes new information on the physiopathology of white dot syndromes and inflammatory chorioretinopathies, notably on whether or not the choriocapillaris is involved. Comparing the OCT-A features allowed us to suggest that both APMPPE and SLC might be part of the same spectrum of inflammatory disease with primary involvement at the level of the choriocapillaris and secondary RPE damage.

To investigate which cytokines, chemokines and growth factors are involved in the immunopathogenesis of idiopathic uveitis, and whether cytokine profiles are associated with. Serum and aqueous humor (AH) samples of 75 patients with idiopathic uveitis were analyzed by multiplex immunoassay. Infectious controls consisted of 16 patients with ocular toxoplasmosis all confirmed by intraocular fluid analyses. Noninfectious controls consisted of 7 patients with Behçet disease related uveitis and 15 patients with sarcoidosis related uveitis. The control group consisted of AH and serum samples from 47 noninflammatory control patients with age-related cataract. In each sample, 27 immune mediators ± IL-21 and IL-23 were measured. In idiopathic uveitis, 13 of the 29 mediators, including most proinflammatory and vascular mediators such as IL-6, IL-8, IL-12, G-CSF, GM-CSF, MCP-1, IP-10, TNF-α and VEGF, were significantly elevated in the aqueous humor when compared to all controls. Moreover, IL-17, IP-10, and IL-21, were significantly elevated in the serum when compared to all controls. We clustered 4 subgroups of idiopathic uveitis using a statistical analysis of hierarchical unsupervised classification, characterized by the order of magnitude of concentrations of intraocular cytokines. The pathogenesis of idiopathic uveitis is characterized by the presence of predominantly proinflammatory cytokines and chemokines and vascular endothelial growth factor with high expression levels as compared to other causes of uveitis. There are indications for obvious Th-1/ IL21-Th17 pathways but also IL9-Th9 and increased IFN-γ-inducing cytokine (IL12) and IFN-γ-inducible CXC chemokine (IP-10). The combined data suggest that immune mediator expression is different among idiopathic uveitis. This study suggests various clusters among the idiopathic uveitis group rather than one specific uveitis entity.

International audience; PURPOSE:To better characterize IgG4-related disease (RD) in the setting of idiopathic orbital inflammation syndrome (IOIS).METHODS:National, multicentre, prospective, observational cohort study. Among the patients consecutively included in the French multicentre SIOI cohort, we selected those who underwent orbital and/or adnexal biopsy. Clinical, morphological and pathological findings at diagnosis were blindly analysed. Serum IgG4 levels at inclusion were measured and all available biopsy specimens were immunostained for IgG4 and IgG. Biopsy samples with more than 10 IgG4-positive plasma cells per high-power field and a IgG4+/IgG+ plasma cell ratio above 40% were scored as positive. IgG4-positive patients were then screened for comprehensive diagnostic criteria for IgG4-RD.RESULTS:Of the 87 patients included, 35 had histologically documented IOIS. Thirteen patients (37%) with a mean age at onset of 27 years (range 21-78) had IgG4-positive biopsies, among which 10 patients (77%) and 3 (23%, with IgG4 serum levels >1.35 g/L) were considered as having probable and definite IgG4-RD, respectively. The latter 13 patients more frequently fulfilled histological criteria for IgG4-RD (including plasmacytic infiltrate (p = 0.006), fibrosis (p = 0.0025) and periphlebitis (p = 0.075)) than IgG4-negative patients. Storiform fibrosis was exclusively found in orbital tissues from IgG4-positive patients (n = 3, 23%). Eosinophilia associated with recurrent sinusitis or asthma was a prominent feature in patients with definite IgG4-RD.CONCLUSIONS:More than one-third of patients with biopsy-proven IOIS satisfied criteria for IgG4-RD, but only a few had a definite type.

The risk of blindness, due to acute ischemic ocular events, is the most feared complication of giant cell arteritis (GCA) since the middle of the 20th century. A decrease of its rate has occurred after the advent of corticoid therapy for this vasculitis, but it seems to have stabilized since then. Early diagnosis and treatment of GCA is key to reducing its ocular morbidity. However, it is not uncommon for ophthalmological manifestations to inaugurate the disease, and the biological inflammatory reaction may be mild, making its diagnosis more challenging. In recent years, vascular imaging has opened up new possibilities for the rapid diagnosis of GCA, and ultrasound has taken a central place in fast-track diagnostic processes. Corticosteroid therapy remains the cornerstone of treatment and must begin immediately in patients with visual symptoms and suspicion of GCA. In that situation, the administration route of corticotherapy, intravenous or oral, is less important than its speed of delivery, any hour of delay worsening the prognosis.

International audience; [u]PURPOSE:[/u] 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. [u]METHODS:[/u] 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. [u]RESULTS:[/u] 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(R) (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. [u]CONCLUSION:[/u] 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.

Purpose To identify predictors of treatment success in syphilitic uveitis (SU). Design Retrospective multicentric analysis of patients treated for SU. Participants A total of 95 eyes (66 patients, mean [standard deviation] aged 49 [12.5] years, 31 [47%] of whom were human immunodeficiency virus [HIV]+) were analyzed. Methods Activity of SU was assessed at 1 week and 1 month after treatment onset, and at last follow-up. Improvement was defined by a ≥2-step decrease of both anterior chamber and vitreous haze inflammation levels, and by the size reduction in chorioretinal lesions. Main Outcome Measures Recovery was defined as the resolution of inflammation in all anatomic structures at 1 month. Results Panuveitis and posterior uveitis were the most frequent findings. Inflammatory parameters were higher in HIV+ patients. Recovery was reported in 65% and 85% of eyes at 1 month and at last follow-up, respectively. In multivariate analysis, after adjusting for initial best-corrected visual acuity and the antimicrobial treatment regimen, clinical improvement at 1 week (corrected risk ratios [cRR], 3.5 [2.3–3.8]; P = 0.001) was predictive of recovery at 1 month, whereas the use of periocular dexamethasone injections (cRR, 0.05 [0.02–0.6]; P = 0.01) and methylprednisolone pulses negatively affected the outcomes of eyes. Conclusions Early improvement is the strongest predictor of ophthalmological recovery in SU.

Introduction L’objectif de cette etude etait d’evaluer de maniere prospective les consequences financieres d’une approche diagnostique standardisee par rapport a une strategie ouverte pour le diagnostic etiologique des uveites. Patients et methodes L’etude ULISSE (PHRC 2009) etait un essai comparatif randomise, de non-inferiorite, prospectif et multicentrique. Les patients consecutifs atteints d’uveite, suivis ou consultant dans l’un des departements d’ophtalmologie des centres participants, ont ete inclus. Dans le groupe standardise, tous les patients presentaient un bilan minimal, quel que soit le type d’uveite (numeration globulaire complete, taux de sedimentation erythrocytaire, proteine C reactive, test cutane a la tuberculine, serologie de la syphilis et radiographie pulmonaire), suivis d’investigations complementaires plus complexes en fonction des constatations ophtalmologiques (type anatomique, lateralite, caractere granulomateux, evolution, presence de signes specifiques). Dans le groupe ouvert, l’ophtalmologiste pouvait ordonner n’importe quel type d’examens complementaires. Le resultat principal etait le cout moyen par patient de chaque strategie. Resultats Neuf cent trois patients ont ete inclus entre janvier 2010 et mai 2013. Le cout moyen par patient de la strategie standardisee etait de 182,97 euros [IC95 % (173,14 ; 192,80)] et le cout moyen par patient de la strategie ouverte etait de 251,75 euros [IC95 % (229,24 ; 274,25)]. Par consequent, le cout moyen par patient de la strategie standardisee etait significativement inferieur au cout moyen par patient de la strategie ouverte (p Conclusion La strategie standardisee, bien que non-inferieure a la strategie ouverte en termes de rentabilite diagnostique, permet des economies significatives pour le diagnostic etiologique des uveites.

Resume Le tableau d’hemorragies retiniennes recidivantes chez de jeunes adultes a ete decrit pour la premiere fois en 1880 par Henry Eales. Les signes associes d’inflammation oculaire ont ete rapportes 5 ans plus tard. La maladie de Eales correspond a un tableau de vascularite retinienne ischemique qui atteint en priorite la retine peripherique des jeunes patients. La majorite des cas ont ete rapportes en Inde. Son etiologie est inconnue mais elle semblerait dans certains cas etre liee a une hypersensibilite mediee par le systeme immun a l’antigene Mycobacterium tuberculosis (MTB). Sa pathogenese est reliee a une ischemie retinienne extensive secondaire a une vasculopathie occlusive avec liberation de facteurs angiogeniques de type VEGF. Les atteintes sont retiniennes et comprennent des periphlebites, une ischemie retinienne capillaire principalement en peripherie pouvant se compliquer de retinopathie proliferante avec neovascularisation retinienne et/ou papillaire, d’hemorragies intravitreennes recidivantes et de decollements de la retine tractionnels. Ces complications peuvent mettre en jeu le pronostic visuel. L’histoire naturelle de la maladie de Eales est assez variable avec une remission temporaire ou permanente dans certains cas et une progression continue pour d’autres. L’evolution se fait vers la bilateralisation de la maladie frequemment ce qui necessite un suivi regulier. Le traitement de la maladie de Eales depend du stade de la maladie et n’est pas bien defini. L’observation simple, la chirurgie par vitrectomie et/ou les injections intravitreennes de VEGF font partie de la prise en charge d’une hemorragie intravitreenne dans ce contexte, en association a la corticotherapie si une vascularite retinienne est presente. Une pan-photocoagulation retinienne par laser est necessaire en cas de neovascularisation retinienne.

To evaluate the efficacy and safety of dexamethasone (DEX) implant compared with inferior fornix-based sub-Tenon triamcinolone injection (PSTA) for treatment of uveitis.A total of 48 eyes received DEX and 49 eyes received PSTA as the first treatment.A total of 31 eyes were implanted with DEX relapsed (64.5%) after the first injection, while 32 eyes were injected with PSTA as the first treatment relapsed (65.3%). Kaplan-Meier estimated survival to overall relapse after the first injection was a mean 20 months± 3.6 months for DEX (median,7) and 14 months± 1.9 months (median,9) for the PSTA (P = 0.505). Of 49 eyes receiving the PSTA implant as the first treatment, inflammation persisted in 14.3% after the first injection but persisted in none after the DEX injection (P = 0.005).DEX implantation achieved a higher rate of disease control in the initial 12 weeks postinjection with a relative equivalence in the duration of effect and relapse rates when compared with PSTA.

We describe the frequency, demographic and clinical features, and visual outcomes of ocular syphilis infections observed during 2012-2015 at a tertiary reference center in Paris, France. Twenty-one cases (29 eyes) were identified. The occurrence of ocular syphilis increased from 1 case in 2012 to 5 cases in 2013, 6 cases in 2014, and 9 cases in 2015 (2.22-25.21/1,000 individual patients/year for the period). Among case-patients, an annual 20%-33% were co-infected with HIV. Seventy-six percent of ocular syphilis infections occurred in men who have sex with men. Seventy-five percent of case-patients had a good final visual outcome (best-corrected visual acuity >0.3 logMAR score). Visual outcome was worse for HIV-positive patients than for HIV-negative patients (p = 0.0139). At follow-up, the best visual outcomes were observed in patients whose mean time from first ocular symptom to consultation was 15 days (SD +19 days).