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Purpose: Birdshot 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., Methods: 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., Results: 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)., Conclusion: 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.

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.

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.

Purpose: Uveitis can be associated with meningitis (uveomeningitis) and the inflammation shared with the central nervous system. We aimed to describe the characteristics and outcome of uveomeningitis., Methods: We retrospectively analyzed 110 consecutive adult patients with uveomeningitis., Results: The main causes of uveomeningitis were Vogt-Koyanagi-Harada (31%), syphilis (16%), sarcoidosis (12%), Behçet's disease (7%), and multiple sclerosis (5%). Sixteen percent of uveomeningitis remained of undetermined origin. Compared to etiology-matched uveitis without meningitis, patients with uveomeningitis were younger, had more frequent neurological manifestations, and had more frequent abnormal cerebral magnetic resonance imaging findings. In contrast, no ocular feature upon examination was significantly associated with the presence of meningitis. Patients with uveomeningitis were more frequently treated with immunosuppressants but uveitis relapse and systemic complications did not differ between groups., Conclusion: Uveomeningitis is associated with a limited spectrum of diseases. Meningitis does not seem to impact ocular and extraocular outcomes. Therefore, lumbar puncture should be performed on an individual basis during the diagnostic workup of uveitis.

Purpose : To assess the choroidal vascularity index (CVI) in birdshot chorioretinopathy (BSCR) patients. Methods : The CVI was calculated as the ratio of luminar area to the total subfoveal choroidal area on enhanced-depth imaging optical coherence tomography. Intraocular inflammation was defined by the presence of macular edema and/or vitritis and/or vasculitis and/or papillitis. Results : Eighty BSCR patients and 22 healthy controls were included. Foveal choroidal thickness was lower in the BSCR compared with the control group ( p < 0.001). CVI was not different between the two groups. CVI was significantly higher in BSCR patients with inflammatory activity ( p = 0.004). Using a multivariate linear regression model, longer disease duration was associated with lower CVI ( p = 0.038), whereas the presence of papillitis was associated with a higher CVI ( p = 0.003). Conclusions : The CVI was not different between the BSCR and the control group. However, the CVI may be used as a new tool for monitoring inflammatory activity in BSCR.

Background : This study aimed to assess the diagnostic relevance of CD4/CD8 ratio in cerebrospinal fluid (CSF) for the etiological diagnosis work-up of uveitis. Methods : We consecutively included patients who were referred to our department for the diagnostic workup of intermediate and/or posterior uveitis. Etiological diagnoses were established in a blind manner regarding CD4/CD8 ratio. Results : Fifty-two patients were included. A diagnosis of ocular sarcoidosis was made in 15 (29%) patients, 21% had another determined diagnosis while 50% remained of undetermined origin. Median CD4/CD8 ratio in CSF was 4.57 (IQR 3.39-5.47) in ocular sarcoidosis, 1.74 (1.60-3.18) in uveitis due to other determined cause ( P = .008), and 2.83 (2.34-3.54) in those with uveitis of undetermined origin ( P = .007). CD4/CD8 ratio >3.23 was associated with a diagnosis of ocular sarcoidosis. Conclusion : Determination of CD4/CD8 ratio in CSF can be useful for diagnosis work-up since a CD4/CD8 ratio >3.23 in CSF is associated with ocular sarcoidosis.

Purpose : The diagnostic workup of uveitis is challenging, with 30 to 50% of cases remaining of undetermined etiology despite multiple investigations. Sarcoid granuloma-related increase of 1,25(OH) 2 D levels could be helpful for the diagnosis of ocular sarcoidosis. Methods : Monocentric retrospective cohort study of patients for whom serum 25(OH)D and 1,25(OH) 2 D levels were measured during the etiologic workup of unexplained uveitis in a tertiary referral center. The diagnoses of uveitis' underlying diseases were established according to international diagnostic criteria. Results : Fifty-nine patients were included. The diagnosis of defined, presumed or probable sarcoidosis was made in 37% of patients while 41% of cases remained of undetermined origin. The median serum levels of 25(OH)D in patients with ocular sarcoidosis and in those with uveitis due to another cause were 34.50 [21.2-40.8] and 43.20 [32.2-58.3] nmol/L (P=0.02), respectively. In the same subgroups of patients, the median serum levels of 1,25(OH) 2 D were 132.4 [107.4-163.9] and 108.0 [84.30-130.5] pmol/l (P=0.02), and the median 1,25(OH) 2 D/25(OH)D ratio was 4.17 [3.11-5.09] and 2.56 [1.54-3.37] (P=0.0007) respectively. A 1,25(OH) 2 D/25(OH)D ratio >3.5 was associated with the diagnosis of sarcoidosis with a 68 % sensitivity and a 78% specificity and, in univariate analysis, was associated with an abnormal chest CT-scan (OR=5.7, P=0.003), granulomas on bronchial biopsy (OR=14.7, P=0.007) and bronchoalveolar lavage fluid lymphocytosis (OR=12.4, P=0.0006). Conclusion : The measurement of serum 25(OH)D and 1,25(OH) 2 D levels is a useful tool in the etiological workup of patients with unexplained uveitis, since a high 1,25(OH) 2 D/25(OH)D ratio is suggestive of ocular sarcoidosis.

Purpose: This study aimed at reporting lymphocytic meningitis in patients diagnosed with sympathetic ophthalmia (SO)., Methods: In this single-center retrospective observational case series, we reviewed cases diagnosed with SO. We analyzed the patients' inciting injuries, the characteristics of uveitis and the cerebrospinal fluid (CSF) analyses., Results: Nine patients were diagnosed with SO and CSF analyses were available in all cases. Four cases had lymphocytic pleocytosis, 3 of which showed marked CSF inflammation with more than 300 lymphocytes/mm 3 . The inciting event in these 3 patients was a globe perforation injury, whereas 4 patients without meningitis had SO following a surgical intervention., Conclusions: In this case series of patients with SO, lymphocytic meningitis was a common finding. The prevalence of meningitis in patients with SO and its value for the diagnosis of the disease needs to be further studied.

Birdshot chorioretinopathy primarily affects patients of European descent. At least 96%, if not all patients, are HLA-A29 carriers. HLA-A*29:01 and HLA-A*29:02, the two main subtypes of HLA-A29, differ only by a single mutation. In the general population HLA-A*29:02 is most frequent in whites, while HLA-A*29:01 is more frequent in Asians. The differential distribution of HLA-A*29:01 and HLA-A*29:02 has been actively debated as an explanation for the selective development of the disease in patients of European descent, but is no longer a valid argument. Another factor, probably not HLA linked, is either protective in Asians and in Africans or, conversely, triggers an autoimmune reactivity that is possibly present in whites and absent in Asians and in Africans. HLA-A*29:02 transgenic mice in which a spontaneous posterior uveitis is observed after 6 months of age provide further evidence that the HLA-A29 molecule plays a role in the pathogenesis of the disease.