Behcet’s Disease

Defined as a systemic vasculitis developing in a particular genetic background, uveitis is one of the hallmarks to diagnose Behcet’s disease and also one of the important clinical criteria to start systemic treatment. Isolated anterior non granulomatous uveitis with hypopyon, even though a classic clinical picture, actually develops in a minority of cases. In most patients, uveitis is posterior, associated to small vessel occlusive retinal vasculitis, carrying a high risk of permanent retinal damage and subsequent severe visual loss. The guarded natural prognosis of the disease has positively changed in the last decennials with the introduction of biologic immunosuppressant agents in the field of uveitis. Vision can be preserved in most cases provided a prompt early diagnosis and adequate therapy. The potential role of oral bacteria as a triggering factor for autoinflammation in predisposed hosts is interesting, opening the door to prevention in this still not well-understood severe uveitis

Acute Posterior Multifocal Placoid Pigment Epitheliopathy as the Initial Manifestation of Sarcoidosis

Purpose: To report an undiagnosed case of systemic sarcoidosis manifesting with bilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Case Report: A 26-year-old Caucasian man was referred for management of unilateral visual loss together with a paracentral scotoma developing 2 weeks after a flu-like syndrome. Clinical signs and ancillary diagnostic investigations suggested APMPPE. Laboratory tests demonstrated elevated serum angiotensin converting enzyme and lysozyme levels. Chest CT-scan disclosed moderate hilar lymph node calcifications but QuantiFERON-TB gold test was negative and bronchoalveolar lavage and biopsies were unremarkable. Accessory salivary gland biopsy disclosed epithelioid and gigantocellular granuloma formation without caseum, confirming a diagnosis of sarcoidosis. The fellow eye was involved a few days later and the patient complained of dyspnea. Echocardiography disclosed severe granulomatous myocardial infiltration and high dose corticosteroids and intravenous cyclophosphamide were initiated. Systemic treatment controlled both cardiac and ocular lesions, and was tapered accordingly. Conclusion: The constellation of "white dot syndromes" and systemic symptoms necessitates a general work-up to exclude granulomatous disorders such as sarcoidosis or tuberculosis. Delayed diagnosis of cardiac sarcoidosis may have life-threatening consequences and the ophthalmologist may be the first physician to diagnose the condition

Use of Fundus Autofluorescence Combined with Optical Coherence Tomography for Diagnose of Geographic Atrophy in Age-Related Macular Degeneration

The aim of this study was to demonstrate the sensitivity of Optical coherence tomography (OCT) in detection of geographic atrophy (GA) secondary to exudative age related macular degeneration (AMD). In this retrospective case series study 77 patients (53% female, with mean ± standard deviation [SD] of 82.6±9.3 years) with 97 eyes (45 OS [left eyes]/52 OD [right eyes]) were included. This was a retrospective review of the charts of patients who presented with exudative AMD at the Pitié Salpetrière Hospital, Paris, France, between December 2016 and August 2017 that received intravitreal injections of anti-vascular endothelial growth factor (anti-VEGF) therapies. At baseline, following biomicroscopy examination, multimodal imaging was performed including, fluorescein angiography (FA), fundus auto-fluorescence (FAF), spectral domain optical coherence tomography (SD-OCT) and indocyanine green angiography (ICGA). During the follow-up, SD-OCT with/without FAF and FA were performed for each patient at 6, 12 and 18 months. For investigation of the prevalence of GA in eyes undergoing intravitreal injections with anti-VEGF therapy, FAF and SD-OCT images were qualitatively reviewed by four independent observers (two graders per group). Kappa coefficient of Cohen was calculated to determine agreement between the graders. The kappa coefficient of Cohen, for inter-rater agreement in the evaluation of FAF images was 0.468, indicating a moderate agreement between the first and second raters. Thus, the sensitivity and specificity of FAF for the diagnosis of GA were 70% and 57%, respectively. If atrophy was assessed with SD-OCT image analysis, the kappa coefficient for inter-rater agreement was 0.846, implying an acceptable agreement between both readers. The sensitivity and specificity of SD-OCT were 93% and 58% respectively. In conclusion, SD-OCT image analysis was more sensitive than FAF for identifying GA in patients treated for exudative AMD. Epub: October 1, 2019

Association between visual function response and reduction of inflammation in noninfectious uveitis of the posterior segment

PURPOSE. To examine the association between visual function response (VFR) and inflammation reduction in active noninfectious uveitis of the posterior segment (NIU-PS). METHODS. Phase 3 SAKURA Study 1 randomized 347 subjects in a double-masked fashion to receive injections of intravitreal sirolimus 44 mu g (n = 117); 440 mu g (n = 114); or 880 mu g (n = 116) every other month. Vitreous haze (VH) response, a measure of inflammation reduction, was defined as a VH score of 0 or 0.5+ at month 5 based on the modified Standardized Uveitis Nomenclature Scale. Visual function was assessed with best-corrected visual acuity (BCVA) and the National Eye Institute (NEI) Visual Function Questionnaire-25 (VFQ-25). In this post-hoc analysis, principal component analysis was used to reduce the information in the multidimensional visual function outcome to a restricted number of independently relevant VFR measures. Minimal clinically important differences (MCID) for the VFQ-25-derived components were based on the standard error of measurements. Overall VFR was defined as either a BCVA improvement of >= 2 lines, or an improvement exceeding the MCID in the VFQ-25 based visual function measures. RESULTS. The VFQ-25 composite score (VFQCS) and mental health subscale score (VFQMHS) were retained as relevant VFRs, with MCIDs of 4.3 and 11.7 points, respectively. A vitreous haze response was significantly associated with each VFR measure: VFQCS (odds ratio [ OR] = 2.23; P = 0.0004); VFQMHS (OR = 2.84; P < 0.0001); BCVA (OR = 2.60; P = 0.0009), and overall VFR (OR = 2.65; P < 0.0001). CONCLUSIONS. Inflammation reduction to a VH score of 0 or 0.5+ was significantly associated with improved visual function. Achieving a VH response of 0 or 0.5+ is a patient-relevant outcome

predictive factors of intraocular pressure level evolution over time and glaucoma severity in fuchs heterochromic iridocyclitis

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Biologic Therapy for HLA-B27-associated Ocular Disorders

The treatment of articular and extra-articular manifestations associated with HLA-B27 has undergone dramatic changes over the past two decades, mainly as a consequence of the introduction of biologic agents and in particular anti-tumor necrosis factor α (anti-TNFα) agents. Uveitis is known to be the most frequent extra-articular feature in HLA-B27-associated spondyloarthritides. Topical corticosteroids and cycloplegic agents remain the cornerstones of treatment. However, biologic therapy may be effective in the management of refractory or recurrent forms of uveitis. This review gives an update on the management of HLA-B27-associated ocular disorders with biologics, including anti-TNFα agents and non-anti-TNFα biologic modifier drugs. There is an emerging role for newer biologics targeting interleukin-12/23 and interleukin-17 for the treatment of spondyloarthritides but data on their efficacy on anterior uveitis are sparse

Uvéite de l'enfant et arthrite juvénile idiopathique

AIX-MARSEILLE2-BU Méd/Odontol. (130552103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Evolution péjorative des pseudo uvéites chroniques lymphomateuses associées à une localisation cérébrale

Introduction : L'incidence et la mortalité du lymphome oculocérébral chez les patients immunocompétents est en progression. La présentation clinique est le plus souvent non spécifique et le diagnostic retardé. Le pronostic fonctionnel et vital demeure réservé. Patients et méthodes : L'étude a porté sur les données rétrospectives cliniques, paracliniques et évolutives de 11 cas de lymphome oculocérébral diagnostiqués à l'hôpital de la Pitié Salpétrière et de Lausanne entre 1991 et 2003. Résultats : 8 hommes et 3 femmes, âgés de 49,4 ans (34 ans à 65 ans) au moment du diagnostic ont été inclus. Les hommes étaient touchés en moyenne 12 ans avant les femmes. Une hyalite bilatérale et corticorésistante était présente dans tous les cas associés à des signes non spécifiques ou plus évocateurs (bouée vitréenne périphérique ou infiltrats sous rétiniens). Le délai entre les signes ophtalmologiques et neurologiques était en moyenne d'un an. Le diagnostic de lymphome à grande cellules B a été confirmé par biopsie cérébrale, et/ou vitréenne ou irienne. Le taux d'IL-10 intra-oculaire était élevé (300 pg/ml en moyenne). Après prise en charge oncologique, 4 patients de sexe masculin sont décédés, 3 ans après les premiers symptômes et 2 ans après le diagnostic. Quatre autres patients sont stabilisés dont 3 avec des séquelles neurologiques et/ou ophtalmologiques invalidantes. Les autres patients ont été perdus de vue. Discussion et conclusions : Si cette affection est classiquement décrite chez la femme durant la 5ème décennie, cette étude souligne la précocité et la gravité de l'affection chez l'homme jeune. Devant un tableau d'uvéite inhabituelle, le diagnostic doit être sytématiquement évoqué et recherché par la répétition des investigations (IRM, dosage de l'IL-10 et analyse cytologique). La précocité de celui-ci représente un facteur pronostique important étant donné l'évolution spontatément mortelle de l'atteinte cérébrale qui semble plus péjorative chez l'homme jeune.PARIS7-Villemin (751102101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

PRISE EN CHARGE DIAGNOSTIQUE ET THERAPEUTIQUE DES PSEUDO NECROSES RETINIENNES AIGUES (DES OPHTALMOLOGIE)

AIX-MARSEILLE2-BU Méd/Odontol. (130552103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF