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Objectives To describe characteristics, treatment, and outcome of isolated antineutrophil cytoplasm antibody (ANCA)-associated scleritis at diagnosis, in comparison with idiopathic scleritis with negative ANCA tests. Methods This retrospective case control multicentre study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data of patients with scleritis without any systemic manifestation and with positive ANCA results were compared to those of a control group of patients with idiopathic scleritis with negative ANCA tests. Results 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022, were included. Median follow-up was 28 months (IQR 10–60). Median age at diagnosis was 48 (IQR 33–60) and 75% were female subjects. Scleromalacia was more frequent in ANCA-positive patients (p = 0.027). 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% versus 34%, p 5 mg/L at diagnosis was the only significant risk factor of progression to systemic AAV (adjusted HR 5.85; 95%CI 1.10–31.01; p = 0.038). Conclusion Isolated ANCA-associated scleritis are mostly anterior scleritis with higher risk of scleromalacia than ANCA-negative idiopathic scleritis and are more often difficult-to-treat. One third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV.

To evaluate mean change in best-corrected visual acuity (BCVA) at 52 weeks in patients with inflammatory choroidal neovascularization (CNV) treated with aflibercept.We conducted a prospective non-comparative open-label trial. Following one mandatory intravitreal injection of aflibercept, patients were treated under a pro re nata (PRN) dosing regimen with monthly visits.A total of 19 patients were included, but one presented exclusion criteria; 16 patients were followed for the whole 52-week study, and data for the primary endpoint analysis were available for 14. At baseline, mean BCVA and mean central retinal thickness (CRT) were 64.53 (±19.64) letters and 351.79 (±97.77) μm, respectively. At 52 weeks, the mean change in BCVA was +9.50 (±12.90) letters [95%CI = +2.05-+16.95]. One patient had lost more than 15-letters at 24 weeks, and another one at 52 weeks. CRT change was -62.77 (±100.73) μm at 24 weeks and -66.53 (±97.47) μm at 52 weeks. There was a mean number of 3.56 (±3.29) intravitreal injections at 52 weeks (min = 1; max = 12). No serious ocular adverse events related to the treatment were reported.Our study shows that aflibercept is clinically effective, both anatomically and functionally in the treatment of inflammatory CNV. Following the first injection, the PRN strategy appears sufficient for treating most choroidal neovessels.

EE cases were retrospectively studied between January 2014 and January 2021. KP EE cases were analyzed to assess clinical, microbiological features, and outcome.Among the 33 EE cases identified, the first causative agent (24%,KP is an emerging cause of EE in a French center, consistently associated with liver abscesses, frequent cerebral involvement, and predominance of hvKP strains.

Introduction Belatacept is associated with a higher incidence of cytomegalovirus (CMV) disease and atypical presentations. Ocular manifestations are rare, representing up to 5% of disease manifestations and previous cases consisted in isolated retinitis. Case Description Herein, we report the case of an 81-year-old kidney transplant recipient who developed an anterior and intermediate uveitis under belatacept therapy. The diagnosis was established using quantitative CMV polymerase chain reaction assays in the aqueous humor. Belatacept was interrupted and oral and topical valganciclovir treatments were instituted. Lesions however extended, leading to intensify the treatment by intra-venous and intra-vitreal ganciclovir injections. Visual acuity stabilized and ocular inflammation was finally controlled after 3 months. Conclusion Clinicians should be aware of CMV infection as a cause of anterior uveitis under belatacept-based regimen, even in the absence of symptoms suggestive of systemic CMV replication.

BRAF inhibitors (BRAFi) and MEK inhibitors (MEKi) significantly improved metastatic melanoma prognosis. Ocular adverse effects (OAEs) represent an uncommon but disabling toxicity of these drugs. We aimed to characterize the ocular safety profile of BRAFi or MEKi and to detect possible safety signals.We performed a retrospective, observational, pharmacovigilance study using VigiBase, the World Health Organization global safety database. Ocular adverse effects were classified according to the eye segments and the inflammatory pattern based on the Standardization of Uveitis Nomenclature. Associations among BRAFi monotherapy, MEKi monotherapy, and BRAFi+MEKi combination therapy and OAE reporting were assessed using disproportionality analysis. Results were expressed with the reporting odds ratio (ROR) and its 95% confidence interval (CI).From January 2010 to October 2019, 1568 OAE cases were reported with BRAFi or MEKi. Among them, 1006 cases with sufficient data were included, corresponding to 310 (30.8%), 124 (12.3%), and 572 (56.9%) cases reported with BRAFi, MEKi, or BRAFi+MEKi combination therapy, respectively. BRAF inhibitor monotherapy was significantly associated with the reporting of iris and ciliary body abnormalities (ROR, 8.7; 95% CI, 6.0-12.5), diffuse abnormalities (ROR, 7.1; 95% CI, 5.4-9.4), anterior uveitis (ROR, 8.6; 95% CI, 6.0-12.1), and panuveitis (ROR, 7.1; 95% CI, 5.4-9.4). MEK inhibitor monotherapy was associated with the reporting of retinal and choroid abnormalities (ROR, 9.5; 95% CI, 7.4-12.2), diffuse abnormalities (ROR, 2.5; 95% CI, 1.1-6.1), and panuveitis (ROR, 2.5; 95% CI, 1.1-6.1). Combinations of BRAFi and MEKi therapies were associated with OAEs from both drugs, with a possible synergistic or additive effect for diffuse abnormalities and panuveitis.Our study characterizes the ocular safety profile of BRAFi and MEKi. We identify possible safety signals for several OAEs not previously reported with BRAFi and MEKi. Our data provide the rationale for a personalized management of OAE in patients with BRAFi+MEKi combination therapy according to the type of ocular reaction.

Non-infectious uveitis is a heterogenous group of potentially blinding ocular autoimmune diseases that may represent a manifestation of a systemic condition or may affect the eyes only. A systemically administered anti-TNF has recently been approved for the treatment of non-infectious uveitis, broadening the therapeutic arsenal available to control intraocular inflammation and reduce uveitis complications that can lead to vision loss. When uveitis affects only the eyes, a local anti-TNF-α administration strategy could optimize the ocular therapeutic effect and reduce undesirable systemic side-effects. A new ocular method of non-viral gene therapy, currently in development, may broaden the indications for ocular anti-TNF-α agents, not only for uveitis but also for other diseases in which TNF-α-mediated neuro-inflammation has been demonstrated.Les anti-TNF-α pour le traitement des uvéites non infectieuses.Les molécules anti-TNF-α administrés par voie générale ont été approuvés récemment pour le traitement des uvéites non inflammatoires, élargissant l’arsenal thérapeutique dans le traitement de ces pathologies responsables de cécité évitable si l’inflammation est contrôlée. Quand seul l’œil est atteint, des stratégies d’administration locale permettraient d’optimiser les effets intraoculaires des molécules anti-TNF-α et d’en réduire les effets indésirables. Une nouvelle méthode de thérapie génique non virale, actuellement en développement, pourrait élargir les indications des molécules anti-TNF-α oculaires, non seulement pour les uvéites, mais également pour d’autres maladies dans lesquelles une neuro-inflammation impliquant le TNF-α a été démontrée.

Background: Anterior-segment optical coherence tomography (AS-OCT) is an important tool, helpful to diagnose and manage many corneal conditions, but has not been reported in cases of peripheral ulcerative keratitis (PUK). The aim of this study is to describe AS-OCT findingsin cases of PUK.Methods: Retrospective observational case series of six eyes with a PUK with proven systemic vasculitis. Clinical course, slit-lamp photographs, and AS-OCT findings were the main outcomes.Results: The AS-OCT findings were found to correlate with the ocular disease’s level of activity. In the acute stage, an absence of corneal epithelium, a scrambled appearance of the anterior stroma and a heterogeneous stromal reflectivity were observed. During the reduction of disease level activity, an irregular hyporeflective epithelium, a smoother anterior stroma, and a homogenous hyperreflective stroma were seen. At the healed stage, a filling of the corneal defect by a hyporeflective thick epithelium, the persistence of the hyperreflective underlying stroma, and a demarcation line were observed.Conclusions: AS-OCT provides an assessment of structural changes occurring in PUK, useful for its diagnosis and monitoring.

Primary open-angle glaucoma (POAG) is a prevalent optic neuropathy with complex genetics. A small number of patients carry a mutation in the coding region of the myocilin (MYOC) gene. The nature and the frequency of these mutations, however, vary substantially, notably with the age at onset and the ethnic origin of the patients. Here, we showed that denaturing high performance liquid chromatography (DHPLC) is an appropriate method for screening carriers of MYOC mutations. We have applied the method to a group of 237 POAG patients and 108 control subjects from France. Mutations were found in 17 (7.5%) patients and in none of the controls. A single mutation, Q368X (c.1102CT), accounted for the majority (12/17) of these mutations, corresponding to a frequency of 5% among POAG patients, the highest ever reported for this mutation. Furthermore, analysis of allelic associations at closely linked microsatellite markers indicated that most, if not all, patients inherited Q368X from a same ancestor. Five other patients carried four distinct mutations, including N480K (c.1440CA) (2 cases), I499F (c.1495AT), G367R (c.1099GA) and T438I (c.1313CT), which is reported here for the first time. Altogether, MYOC mutations in French patients were associated with a significantly increased intraocular pressure at diagnosis. In addition, the age at diagnosis of patients with a mutation other than Q368X was significantly younger than that of Q368X carriers or of patients with a normal MYOC. Based on these observations, a screening strategy of MYOC mutations in French POAG patients is briefly outlined.