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Abstract Background East and South East Asian subjects as well as Amerindians and Hispanic subjects are predominantly affected by Vogt-Koyanagi-Harada disease. In Europe, only few studies have described the clinical features and treatment of this disease, especially in France. Methods This retrospective case series was based on data collected from patients with a VKH disease diagnosed from January 2000 to March 2017, provided by three French Tertiary Centers. Results Forty-one patients (16 men and 25 women) were diagnosed: average age at diagnosis was 38.7 years. Patients were mainly from Maghreb (58%), but ethnic origins were multiple. Pleiocytosis was observed in 19 cases (63%) and 17 out of 41 patients showed audio vestibular signs (41%), and 11 showed skin signs (27%). Thirty-four were treated with corticosteroids (83%), 11 with an immunosuppressant treatment (27%) and 5 with biological therapy drugs (13%). Relapse was observed in 41% patients, even though final average visual acuity had improved. We did not find any significant clinical difference in the population from Maghreb compared to other populations, but for age and sex trends, since there was a majority of younger women. Conclusion We report here the second largest French cohort reported to date to our knowledge. The multiethnicity in our study suggests that VKH disease should be evoked whatever patients’ ethnicity.

Aims: To compare effectiveness of subconjunctival triamcinolone acetonide injections and intravitreal injections of dexamethasone 700 µg implants in reducing central macular thickness (CMT) in uveitic and postoperative macular oedema (ME)., Methods: We conducted an open-label, French multicentre randomised comparative trial with a logarithmic CMT non-inferiority margin set at 0.06. Patients were adults with non-infectious inflammatory ME, without any contraindication to the treatments. They were randomised 1:1 to receive either triamcinolone or dexamethasone. The primary endpoint was the difference in CMT among treated eyes between baseline and 2 months, measured with spectral-domain optical coherence tomography. Secondary outcomes included visual acuity, laser flare, vitreous haze, duration of action, tolerance to injections and adverse events., Results: Between January 2016 and January 2020, 106 patients were enrolled (54 in the triamcinolone group and 52 in the dexamethasone group). Subconjunctival triamcinolone injections seemed to be non-inferior to intravitreal dexamethasone injections, especially at month 3 (and nearly at month 1). Nevertheless, we could not demonstrate it, with a treatment effect at month 2 of 0.05 (0.01 ; 0.09) (p value=0.001). This was corroborated by post hoc analyses in the postoperative subgroup, for whom the non-inferiority was nearly demonstrated at month 2 with a treatment effect of 0.02 (-0.03 ; 0.08) (p=0.37). There was no significant difference in the occurrence of adverse effects., Conclusion: We could not demonstrate the non-inferiority of triamcinolone injections at month 2. Nevertheless, they showed some efficacity, particularly in treating postoperative ME, being as safe as dexamethasone injections, without any loss of chance if a therapeutic switch is necessary., Competing Interests: Competing interests: M-AV: data safety monitoring board member in Comité de Protection des Personnes—Ouest IV since september 2022. Payment for expert testimony by Institut National du Cancer in 2023. BB: grants/contracts with Horus Pharma, AbbVie/Allergan. Consulting fees by Horus Pharma, AbbVie/Allergan, Novartis, Active Biotech, Acelyrin, Roche. Advisory board for Genesight. ML: consulting fees/honoraria/payments for expert testimony by Alcon, Allergan, Bausch & Lomb, DMG, Dompe, Horus Pharma, MSD, Novartis, Quantel, Santen, Shire, Topivert, Théa. Support for attending meetings and/or travel by Bausch & Lomb, Novartis, Théa. M-BR: consulting fees/honoraria/support fort attending meetings by Allergan, Horus Pharma. KA: advisory board in uveitis for Horus Pharma. CChiquet : consulting fees for AbbVie, Amo, Horus Pharma, Théa. Advisory board for Horus Pharma. LK: consulting fees/honoraria by AbbVie, Alimera, Bayer, Novartis, Roche, Théa. Support for attending meetings and/or travel by AbbVie, Bayer, Novartis. Advisory board for Alimera, Bayer, Horus Pharma, Novartis. SB: honoraria by Abbvie, Horus Pharma, Novartis, Roche. Supporting for attending meetings and/or travel by Abbvie, Bayer, Roche. Advisory board for AbbVie, Bayer, Horus Pharma. CPC-G: grants/contracts for the institution with Bayer, Novartis. Consulting fees by AbbVie/Allergan, Alcon, Apellis Pharmaceuticals, Bayer, Horus Pharma, Novartis, Roche, Théa. Honoraria and support for attending meetings and/or travel by Allergan, Bayer, Novartis, Roche. MW: consulting fees/honoraria by AbbVie., (© Author(s) (or their employer(s)) 2025. No commercial re-use. See rights and permissions. Published by BMJ Group.)

Purpose: To describe a patient with hypertensive herpetic uveitis complicated by arterial retinal occlusions and a decompression retinopathy revealing a sickle cell trait., Study Design: Case report., Results: A 24-year-old African man presented with a hypertensive herpetic keratouveitis. A brutal lowering of the intraocular pressure (IOP) by systemic acetazolamide resulted in a ocular decompression retinopathy and multiple arterial occlusions involving the macular and the mid-periphery retina. A hemoglobin electrophoresis revealed a sickle cell trait., Conclusion: Under rare circumstances, vaso occlusive events can occur in patients with a sickle cell trait. We identified high IOP and acetazolamide to be responsible of an increased blood viscosity and a reduction of the vessels' caliber, resulting in sickling and arterial retinal occlusions. We recommend a thorough anamnesis and a sickle cell screening for patients of African or Mediterranean descent with acute elevated IOP, especially if they have to be treated with carbonic anhydrase inhibitors. Abbreviations: HbA: Hemoglobin A; HbS: Hemoglobin S; HSV1: Herpes Simplex Virus - 1; IOP: IntraOcular Pressure; OCT-A: OCT-Angiography; SD-OCT: Spectral Domain Optical Coherence Tomography.

Introduction: Paediatric granulomatous uveitis (PGU) is rare. In addition, lack of awareness often leads to delayed diagnosis and poor visual outcome. Identifying the underlying cause and deciding how best to treat each patient is challenging., Objectives: To evaluate the demographics, aetiologies, complications, treatments, and visual prognosis of paediatric non-infectious granulomatous uveitis., Methods: Retrospective chart review of non-infectious PGU occurring in children before the age of 16 years recruited from the Paediatric Rheumatology Unit, Bicêtre Hospital, France, from 2001 to 2023., Results: We included 50 patients with 90 affected eyes: 29 with idiopathic uveitis, 15 with sarcoidosis, 5 with juvenile idiopathic arthritis, and one with Vogt-Koyanagi-Harada disease. Median age at diagnosis was 9.8 years (range 7.2-12.5). The sex-ratio M/F was 0.52. The most common features of PGU were: panuveitis (56%), bilateral (84%), and chronic (84%). Sarcoidosis was the most frequent diagnosis after idiopathic disease, particularly in the presence of lymphopenia and hypergammaglobulinemia. Uveomeningitis was present in 12% of cases. Upon diagnosis, ocular complications were present in 68 of 90 eyes (76%) particularly in cases of panuveitis. The most commonly used treatments were systemic corticosteroids (72%) and methotrexate (80%). Twenty-three percent of eyes were in remission at last follow-up, 68% were inactive and 4% remained active. The median duration of follow-up was 5.8 years., Conclusion: We report the largest cohort of PGU. PGU were mostly idiopathic and had a high rate of complications. Sarcoid and idiopathic panuveitis are serious illnesses in which disease-modifying therapy should be initiated at diagnosis to improve management., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: To examine whether the COVID-19 pandemic was associated with an increased incidence of uveitis in children., Study Design: We performed a time-series analysis of patient records from a national, hospital-based, French surveillance system. All children hospitalized for uveitis in France between January 2012 and March 2022 were included. The incidence of newly diagnosed uveitis per 100 000 children per trimester in France was analyzed by a quasi-Poisson regression. A cohort of children diagnosed with uveitis at Robert-Debré Hospital was used to compare the characteristics of uveitis after and before the onset of the pandemic., Results: During the study period, 2492 children were hospitalized for uveitis in France. The COVID-19 pandemic, which started in March 2020, was associated with a significant increase in the occurrence of uveitis (estimated cumulative change, 44.9%; 95% CI 11.4-78.4; P < .001). The increase in the incidence of pediatric uveitis started in October 2020, while the national immunization program targeting children aged less than 18 years began in June 2021. This increase involved all forms of uveitis, regardless of location, and clincial characteristics were similar to those diagnosed before the pandemic., Conclusions: Our study evidenced a significant increase in the incidence of pediatric uveitis following the COVID-19 pandemic. This increase occurred 6 months before the implementation of the national COVID-19 vaccination program for children, suggesting that the resurgence of this rare disease is independent of COVID-19 vaccination., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Purpose: To report two cases of multiple evanescent white dot syndrome (MEWDS) following COVID-19 vaccination with the BNT162b2 mRNA vaccine., Methods: Two case reports. Case-1: A 40-yo Caucasian male, complained of blurred and decrease of vision in his left eye (OS) one week after the first dose of the BNT162b2 mRNA SARS-CoV-2 vaccine. Funduscopic examination OS showed multiple granular white dots with an aspect of foveal granularity. Case-2: A 23-yo woman also presented with defective and decrease of vision OS. She received her first dose of the BNT162b2 mRNA SARS-CoV-2 vaccine ten days before. Dilated fundus examination OS showed altered macular reflex with an aspect of foveal granularity., Results: Multimodal imaging showed features of MEWDS in both cases. The anomalies found resolved spontaneously after 6 weeks., Conclusion: Inflammation and immune dysregulation induced by COVID-19 mRNA vaccine or its adjuvants could be involved in ocular adverse effects.

Objective: To highlight the safety and efficacy of Tumor Necrosis Factor inhibitors (anti-TNF) in inflammatory choroidal neovascularization (CNV) in the pediatric population., Design: Retrospective case series., Participants: Three patients, < 16 years old with uveitic inflammatory CNV., Methods: Patients received systemic steroids, methotrexate (MTX), intravitreal (IVT) injections of bevacizumab, and anti-TNF (infliximab or adalimumab) in case of refractory leakage., Results: Five eyes of three pediatric patients (mean age 6 years old) presenting with CNV and put on anti-TNF were followed up for a minimum of 32 months. Four out of five eyes had improved vision, reduced fluid on clinical exam and macular spectral-domain optical coherence tomography (SD-OCT), and cessation of leakage on fundus fluorescein angiography (FFA) after introduction of anti-TNF agents. Two patients developed minor psoriasis treated topically., Conclusion: Anti-TNF agents showed efficacy and safety in a sustainable leakage control of inflammatory pediatric CNV along with improvement in vision.

To suggest a unique missense variant candidate based on long-term ophthalmological changes and associated systemic signs described in five patients from two unrelated families affected by an autosomal dominant multi-systemic disorder including Retinal dystrophy, Optic nerve oedema, Splenomegaly, Anhidrosis and migraine Headaches, called ROSAH syndrome, related to a unique missense variant in ALPK1 gene. Observational longitudinal follow-up study of unrelated families. Clinical analysis of ophthalmological and systemic examinations was performed, followed by genetic analysis, including targeted Next Generation Sequencing (NGS) and Whole-Genome Sequencing (WGS). The ophthalmological phenotype showed extensive optic nerve swelling associated with early macular oedema and vascular leakage. The main associated systemic manifestations were recurrent fever, splenomegaly, anhidrosis, mild cytopenia, anicocytosis and hypersegmented polynuclear cells. WGS, shortened in the second family by the gene candidate suggestion, revealed in all patients the heterozygous missense variant c.710C>T; p.(Thr237Met) in ALPK1. The primary morbidity in ROSAH syndrome in this cohort appeared ophthalmological. Comprehensive, detailed phenotype changes aided by the advancement in genetic testing could allow an early genetic diagnosis of ROSAH syndrome and targeted treatment. The unique missense variant may be suggested as a target of gene correction therapy., (© 2022 The Authors. Clinical Genetics published by John Wiley & Sons Ltd.)

Purpose: To compare the relapse rate of sight-threatening noninfectious uveitis (NIU) in patients treated with infliximab (IFX) or adalimumab (ADA)., Design: Observational retrospective multicenter study., Methods: A total of 330 patients (median age, 36 years; interquartile range, 27-54), 45.2% men) with sight-threatening NIU (ie, retinal vasculitis and/or macular edema) treated with anti-tumor necrosis factor [TNF]-α agents (IFX intravenously at 5 mg/kg at weeks 0, 2, 6, and every 4 to 6 weeks or ADA subcutaneously at 80 mg, then 40 mg every 2 weeks). Data were obtained retrospectively from patients' medical records. Main outcome measures were relapse rate, complete response of NIU, corticosteroid sparing effect, and safety., Results: Main etiologies of uveitis included Behçet disease (27%), idiopathic juvenile arthritis (5.8%), and sarcoidosis (5.5%). The estimated relapse rate at 6 months after introduction of biological agents was 13% (95% CI = 0.009-0.16). IFX was associated with less relapse risk than ADA (hazard ratio [HR] = 0.52, 95% CI = 0.36- 0.77, P = .001). ADA and IFX were comparable in terms of complete response rate of NIU as well as corticosteroid-sparing effect. Behçet disease was associated with higher odds of complete response (HR = 2.04, 95% CI = 1.16 -3.60, P = .01] and lower relapse rate (HR = 0.53, 95% CI = 0.33-0.85, P = .009) than other causes of NIU with anti-TNF-α agents., Conclusions: In sight-threatening NIU, IFX seems to be associated with a lower relapse rate than ADA., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Acute macular neuroretinopathy (AMN) is an increasingly diagnosed disorder associated with several diseases. The aim of this study was to report the incidence of AMN cases diagnosed during the 2020 coronavirus disease 2019 (COVID-19) pandemic year in a French hospital, and to describe their different forms., Methods: All patients diagnosed between 2019 and 2020, in Paris Rothschild Foundation Hospital, with AMN, paracentral acute middle maculopathy (PAMM) and multiple evanescent white dot syndrome (MEWDS) were retrospectively collected using the software Ophtalmoquery ® (Corilus, V1.86.0018, 9050 Gand, Belgium). Systemic and ophthalmological data from AMN patients were analyzed., Results: Eleven patients were diagnosed with AMN in 2020 vs. only one patient reported in 2019. The incidence of AMN significantly increased from 0.66/100,000 visits in 2019 to 8.97/100,000 visits in 2020 ( p = 0.001), whereas the incidence of PAMM and MEWDS remained unchanged. Four (36%) of these AMN patients were tested for COVID-19 and received positive polymerase chain reaction (PCR) tests., Conclusions: The incidence of AMN cases increased significantly in our institution in 2020, which was the year of the COVID-19 pandemic. All AMN-tested patients received a positive COVID PCR test, suggesting a possible causative link. According to the different clinical presentations, AMN may reflect different severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) pathogenic mechanisms.

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.

Objective: Describe our experience with tocilizumab in the treatment of refractory relapsing polychondritis with ocular involvement. Methods: Retrospective consecutive interventional case series that included all patients that received tocilizumab for the treatment of relapsing polychondritis with ocular manifestations. Results: Three cases were selected and the duration of tocilizumab treatment ranged from 1 to 2 years. One of our patients received tocilizumab as a first-line immunosuppressive treatment directly after prednisone. All achieved complete response to tocilizumab 1 month after treatment initiation. No advert events were reported during the follow-up period except for transient neutropenia without any associated infection. Conclusion: Our three cases suggest that tocilizumab may be an effective and safe treatment for ocular manifestation associated with relapsing polychondritis.

Controlling long-term inflammation during non-infectious intermediate, posterior or panuveitis while limiting side effects remains challenging. There is no standardized pre-therapeutic evaluation providing diagnostic certainty, but some simple tests allow us to identifiy the main etiologies. The ophthalmologist identifies the type of uveitis, and the internist completes the investigations according to the ophthalmologist's findings. Fundus photographs, optical coherence tomography, and fluorescein and indocyanine green angiography should be considered during diagnosis and follow-up. Ocular complications of uveitis are numerous. They require close monitoring and specific medical and sometimes surgical management. The growing number of available drugs makes it possible to optimize the management of these conditions with varied etiologies and presentations. Currently, systemic corticosteroids remain the mainstay of therapy, and other alternatives are considered in the case of poor tolerance, steroid resistance or dependence. The choice of a systemic, periocular or intravitreal treatment depends on several factors: chronicity or recurrence of uveitis, duration, bilaterality, association with a systemic inflammatory disease, the presence of contraindications to certain treatments, and also socioeconomic constraints. It is of the utmost importance to find the best compromise allowing tight control of ocular inflammation by means of adapted systemic and/or local treatment while avoiding the main complications., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Rationale: Crystal sorting histiocytosis (CSH) is a rare disorder that is morphologically characterized by the accumulation of monoclonal immunoglobulin crystals, predominantly of a kappa light chain type, within lysosomes of macrophages. CSH may result in a variety of clinical manifestations depending on the involved organs. In this case report, we aim to describe a patient with ophthalmic manifestations which lead to the diagnosis of multiple myeloma with crystal-storing histiocytosis, crystalline podocytopathy, and light chain proximal tubulopathy., Patient Concerns: A 60-year-old male patient presented with progressive bilateral decreased vision for 2 years., Diagnosis: Ophthalmic explorations showed bilateral macular and papillary edema, and multiple crystalline deposits in the anterior stromal cornea and in the retina. Laboratory tests showed nephrotic syndrome and renal dysfunction. Further work-up revealed IgG kappa multiple myeloma, with biopsy-proven combined crystalline podocytopathy and tubulopathy., Interventions: The patient received chemotherapy (bortezomib, cyclophosphamide, and dexamethasone for 3 cycles, then bortezomib, lenalidomide, and dexamethasone)., Outcomes: Despite partial hematologic response and improvement of the papilledema and macular edema, the patient developed dialysis-dependent end-stage renal failure., Lessons: This report, highlighting the protean presentation of paraprotein-mediated injuries, provides additional information on the ocular anomalies not previously described that may be associated with crystal-storing histiocytosis.

Purpose of Review: Checkpoint inhibitors have been increasingly considered as new targets for cancer therapies. Patients receiving checkpoint inhibitors develop many immune-related adverse events (IRAEs). However, ophthalmic IRAEs are rare and have been reported in less than 1% of patients. To date, few case reports evaluating the ophthalmological side-effects of checkpoint inhibitors have been published. In this review, we plan to report the different ocular and orbital side-effects of the checkpoint inhibitors, and to help guide ophthalmologists and oncologists in their management., Recent Findings: Ocular side-effects of checkpoint inhibitors include peripheral ulcerative keratitis, uveitis, Vogt-Koyanagi-Harada syndrome, choroidal neovascularization and melanoma-associated retinopathy. Both thyroid-associated orbitopathy and idiopathic orbital inflammation have also been reported in association with checkpoint inhibitors. Mild IRAE can be treated with topical steroids, whereas systemic corticosteroids and discontinuation of checkpoint inhibitors are indicated in more severe ocular and orbital inflammation., Summary: Physicians involved in the care of oncologic patients should be aware of the ocular and orbital IRAEs that may develop with checkpoint inhibitors. A strong cooperation between oncologists and ophthalmologists is required in the diagnosis and prompt management of these IRAEs.

The authors report the case of a healthy 56-year-old man presenting with bilateral vision loss. Clinical features were consistent with the diagnosis of acute exudative polymorphous vitelliform maculopathy (AEPVM). The patient returned 10 days later with bilateral anterior granulomatous uveitis, and the inflammatory work-up revealed treponemal antibodies in the serum and spinal fluid, consistent with a diagnosis of active neurosyphilis. The patient received standard treatment for neurosyphilis with intravenous penicillin G. Two months later, the intraocular inflammation had resolved, but the resolution of the vitelliform lesions was more gradual. An immune process could be a plausible explanation for these clinical findings. Clinicians should be aware that syphilis can produce AEPVM., (Copyright 2014, SLACK Incorporated.)