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IntroductionEosinophils have widespread procoagulant effects. In daily practice, eosinophil-related cardiovascular toxicity consists of endomyocardial damage, eosinophilic vasculitis and arterial or venous thrombosis. Here we aim to report on the clinical features and treatment outcomes of patients with unexplained ophthalmic vascular manifestations and eosinophilia.MethodsWe conducted a retrospective, multicenter, observational study and a literature review of patients with eosinophilia (≥0.5 x109/L) and concomitant ophthalmic vascular manifestations independent of the underlying eosinophilic disease but with no alternative cause for ophthalmic manifestations. ResultsFifty-seven patients were included (20 from the observational study and 37 from the literature review). Ophthalmic vascular features were the initial manifestation of eosinophil-related disease in 34 (59%) patients and consisted of 29 central retinal artery occlusions, six branch retinal artery occlusions, five central retinal vein occlusions, two branch retinal vein occlusions, seven retinal vasculitides, two retinal vasospasms, 12 Purtscher’s retinopathies, 13 anterior ischemic optic neuropathies and two posterior ischemic optic neuropathies. The median [IQR] absolute eosinophil count at onset of ophthalmic vascular manifestations was 3.5 [1.7-7.8] x109/L. Underlying eosinophil-related diseases included eosinophilic granulomatosis with polyangiitis (n=32), clonal hypereosinophilic syndrome (HES) (n=1), idiopathic HES (n=13), lymphocytic HES (n=2), adverse drug reactions (n=3), parasitosis (n=2), polyarteritis nodosa (n=1), IgG4-related disease (n=1), eosinophilic fasciitis (n=1) and primary sclerosing cholangitis (n=1). Other extra-ophthalmologic arterial or venous thromboses related to eosinophilia were reported in four (7%) and nine (16%) patients, respectively. Visual prognosis was poor: only eight (10%) patients achieved full recovery of ophthalmologic symptoms. After a median follow-up of 10.5 [1-18] months, one patient (3%) had a recurrence of an ophthalmic vascular manifestation, and three patients (10%) had a recurrence of other vascular symptoms (deep vein thrombosis in two and pulmonary embolism in one patient). At the time of recurrence, absolute eosinophil counts were above 0.5 x109/L in all cases (n=4). DiscussionThis study broadens the spectrum of vascular manifestations associated with hypereosinophilia by adding ophthalmic vascular manifestations. In patients with ophthalmological vascular manifestations and hypereosinophilia, aggressive treatment of the underlying pathology (and normalization of blood count) should be implemented.

Purpose: To assess the manifestations of birdshot chorioretinitis (BSCR) in patients aged 80 and over. Design: Among patients with BSCR followed in the CO-BIRD prospective cohort (ClinicalTrials.gov Identifier: NCT05153057), we analyzed the subgroup of patients aged 80 and over. Methods: Patients were assessed in a standardized manner. Confluent atrophy was defined as hypoautofluorescent spots on fundus autofluorescence (FAF). Results: We included 39 (8.8%) of the 442 enrolled CO-BIRD patients. The mean age was 83.8 ± 3.7 years. The mean logMAR BCVA was 0.52 ± 0.76, with 30 patients (76.9%) having 20/40 or better in at least one eye. Thirty-five (89.7%) patients were receiving no treatment. Confluent atrophy in the posterior pole, disrupted retrofoveal ellipsoid zone and choroidal neovascularization were associated with logMAR BCVA >0.3 (p <.0001). Conclusion: In patients aged 80 and over we observed a striking heterogeneity of outcomes, but most retained a BCVA that allowed them to drive. [ABSTRACT FROM AUTHOR]

To assess the effect of corticosteroids (CS) on choroidal neovascularization (CNV) occurrence and recurrence of activity over 2 years in patients with punctate inner choroidopathy (PIC) or multifocal choroiditis (MFC). Retrospective longitudinal study. Previous use of CS was analyzed between group without CNV and group with CNV occurrence and recurrence. Thirty-six patients were included. Patients with CNV were less likely to have received CS in the 6 months following PIC or MFC diagnosis (17% versus 65%, p-value = 0.01). Patients with CNV who had a recurrence of neovascular activity were less likely to have received a previous CS therapy (20% versus 78%; odds ratio = 0.08, p-value = 0.005). This study suggests that patients with PIC and MFC should be treated by CS to prevent CNV development and decrease CNV recurrences. [ABSTRACT FROM AUTHOR]

Purpose: The study aims to determine the impact of initial management in Vogt--Koyanagi--Harada syndrome (VKHS). Methods: Patients diagnosed with a VKHS between January 2001 and December 2020 in two French tertiary centers were included in a retrospective study. Results: Fifty patients were included with a median duration of follow-up of 29.8 months. All patients received oral prednisone after methylprednisolone in all but four of them. Five patients received at least one associated immunosuppressive therapy (IST) within the first 6 months and 26 patients received IST during the entire follow-up period. Twenty-eight patients presented at least one relapse at a median of 5.4 months from diagnosis. Multivariate analyses demonstrated a significant association between relapse and delayed treatment (>26 days) (HR = 3.69, CI95% 1.30--10.47, p = .01), whereas no association was observed between relapse and the number of corticosteroid pulses at initial management. Conclusion: An early corticosteroid treatment within the first 26 days of symptoms decreased the relapse rate. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionBirdshot chorioretinitis (BSCR) is an ocular HLA-related disease with variable clinical progression. We examine the quality of life (QOL) of BSCR individuals aged ≥80 years, providing insights into the long-term disease impact.We utilized data from the CO-BIRD cohort (ClinicalTrials.gov Identifier: NCT05153057) conducted at Hôpital Cochin in Paris, France, focusing on BSCR patients aged ≥80. The main outcome was vision-related QOL using the National Eye Institute Visual Function Questionnaire-25 (NEI VFQ-25). We used Spearman correlation to explore the impact of better BCVA and MD on the composite score, and the Wilcoxon test to evaluate specific visual symptoms’ effects.The study included 35 patients with a mean age of 83.9 ± 3.7 years, 74.3% of whom received systemic immunosuppression. The mean composite score was 58 ± 30, with a median of 75 (23–79). The lowest subscores were driving capacity (38 ± 38), mental health (49 ± 33), and role difficulties (50 ± 35), while the highest were for ocular pain (70 ± 25) and social function (70 ± 38). Decimal BCVA below 0.5 and MD below −6 dB were associated with lower subscores. BCVA and MD were strongly correlated with the composite score (R = 0.67). Symptoms of poor color and blurry vision were significantly associated with lower composite score (p < 0.005).Most BSCR patients over 80 in our cohort maintained sufficient vision for daily activities. The high standard deviation and wide range of VFQ-25 results reflect the heterogeneity of visual outcomes among elderly BSCR patients. [ABSTRACT FROM AUTHOR]

Objectives: To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests., Methods: This retrospective multicentre case-control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests., Results: A total of 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022 were included. The median follow-up was 28 months (IQR 10-60). The median age at diagnosis was 48 years (IQR 33-60) and 75% were females. Scleromalacia was more frequent in ANCA-positive patients (P = 0.027) and 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% vs 34%; P < 0.001), and rituximab (P = 0.03) and had a lower remission rate after the first- and second-line treatment. Systemic ANCA-associated vasculitis (AAV) occurred in 30.7% of patients with PR3- or MPO-ANCA, after a median interval of 30 months (IQR 16.3-44). Increased CRP >5 mg/l at diagnosis was the only significant risk factor of progression to systemic AAV [adjusted hazard ratio 5.85 (95% CI 1.10, 31.01), P = 0.038]., Conclusion: Isolated ANCA-associated scleritis is mostly anterior scleritis with a higher risk of scleromalacia than ANCA-negative idiopathic scleritis and is more often difficult to treat. One-third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)