Ocular syphilis masquerading as CMV retinitis in a transplanted patient.

BACKGROUND: Syphilis has historically been referred to as "the great imitator", for the extent of disease manifestations secondary to infection. Ocular manifestations include a wide range of intra-ocular inflammation. METHODS: In this study, we report the case of a 52 years-old male patient with syphilitic hemorrhagic necrotizing retinitis. RESULTS: The patient presented to the emergency room for rapid and progressive vision loss and ocular redness lasting three weeks and was under immunosuppressive treatment. The diagnosis was syphilitic hemorrhagic necrotizing retinitis mimicking the typical clinical picture of retinitis caused by Cytomegalovirus infection in immunocompromised patients. CONCLUSIONS: The presented case highlights the need to consider ocular syphilis as a great masquerader even in the presence of atypical presentations such as hemorrhagic retinitis. Syphilis should be tested for treponemal and non-treponemal tests, and it should be ruled out as an etiological agent in every case of new-onset intra-ocular inflammation.

Clinical and imaging biomarkers of response to intravitreal dexamethasone implant in eyes with non-infectious uveitic macular oedema.

OBJECTIVE: To investigate clinical and spectral-domain optical coherence tomography (SD-OCT) biomarkers correlating with pre-injection visual acuity (VA), post-injection VA, and the likelihood of macular oedema (MO) regression following dexamethasone (DEX) implant injection in non-infectious uveitic (NIU) patients. METHODS: Patient data from Uveitis Services in Milan, Paris, and Berlin were analysed. Eligible participants were NIU patients aged >18 years with MO as the primary indication for DEX treatment. SD-OCT scans and clinical data were collected at the time of DEX injection (pre-injection visit) and after 3 months (post-injection visit). Multivariable regression models, adjusted for pre-injection VA and lens status, were employed to explore associations. MO regression was defined as the absence of intraretinal/subretinal fluid at the post-injection visit. RESULTS: Our analysis comprised data from 173 DEX treatments, encompassing 103 eyes from 80 patients, with 38 eyes (37%) receiving repeated DEX injections. The absence of the ellipsoid zone (EZ) layer and disorganisation of the inner retinal layers (DRIL) were associated with worse pre- (+0.19 LogMAR, 95% CI 0.01-0.38, p = 0.06, and +0.10 LogMAR, 95% CI 0.02-0.21, p = 0.01) and post-injection VA (+0.33 LogMAR, 95% CI 0.08-0.57, p = 0.01, and +0.17 LogMAR, 95% CI 0.01-0.32, p = 0.04). EZ disruption and DRIL increased significantly (p = 0.01 and p = 0.04), and the chance of gaining ≥5 letters declined in eyes undergoing repeated DEX (p = 0.002). The rate of MO regression after each DEX was 67%. Prolonged MO duration (OR = 0.75/each year, p = 0.02) was associated with reduced likelihood of MO regression. Subretinal fluid was associated with higher rate of MO regression (OR = 6.09, p = 0.01). CONCLUSION: Integrity of the inner and outer retina is associated with better visual response to DEX. Long-standing or recurrent MO is associated with less chance of both visual and anatomic response. Timely treatment is necessary to maximise the outcomes of MO in NIU patients.

Photoreceptor Integrity in MEWDS: Longitudinal Structure-Function Correlations.

Purpose: The purpose of this study was to investigate structure-function correlations in multiple evanescent white dot syndrome (MEWDS) using microperimetry (MP) and spectral-domain optical coherence tomography (SD-OCT). Methods: Single-center prospective observational study including 14 eyes from 13 patients with MEWDS monitored over a median of 49.5 days (interquartile range = 29-92 days). Investigations focused on best-corrected visual acuity (BCVA), foveal granularity, and the Photoreceptor Reflectivity Ratio (PRR) as a measure of photoreceptor integrity. MP assessed average retinal threshold sensitivity (RTS) and bivariate contour ellipse area (BCEA) for fixation stability. A linear mixed model was used to test associations and interactions among RTS, time, and clinical variables. A hierarchical linear mixed model was used to analyze structure-function relationships, addressing both individual and location-specific variations. Results: Overall, 2340 MP locations were tested. PRR revealed a transient decrease within 30 days post-presentation, indicative of early photoreceptor disruption, followed by a progressive increase, signaling recovery. Significantly lower foveal sensitivity (RTS = 14.8 ± 7.4 vs. 22.5 ± 4.4 decibel [dB], P = 0.04) and increased fixation spread (63% BCEA = 1.26 ± 0.97 vs. 0.48 ± 0.35 deg2, P = 0.06) were noted in eyes with foveal granularity compared to those without. A significant increase in RTS was demonstrated over time (0.066 dB/day, P < 0.001), with a central-to-peripheral gradient of improvement. The interaction between follow-up time and baseline BCVA (P < 0.001) indicated more rapid improvement in eyes with worse initial vision. There was a robust, nonlinear association between PRR and RTS across all tested locations (P < 0.001), becoming asymptotic for sensitivity losses exceeding 20 dB. Conclusions: Photoreceptor reflectivity accurately aligned with visual function in MEWDS on longitudinal examinations. The central-to-peripheral gradient of improvement may suggest specific vulnerabilities underlying the area around the disc.

Unraveling the Spectrum of Uveitis: Insights from an Epidemiological Study in a National Referral Center in Northern Italy.

PURPOSE: Uveitis embraces a heterogeneous group of vision-threatening inflammatory conditions. Understanding uveitis epidemiology, etiology, and clinical findings is fundamental for a prompt diagnosis and optimal patient management. The aim of the study is to report the epidemiology of uveitis in a national referral center in Northern Italy and investigate the visual prognosis. METHODS: This retrospective study was conducted at Uveitis Service (Ospedale San Raffaele) between June 2016 and May 2023. Demographic data, clinical characteristics, and etiological diagnoses of uveitis patients were collected, and visual prognosis was longitudinally explored. RESULTS: 1105 patients with uveitis were included in the study, while 47 patients presented neoplastic masquerade syndrome and have been excluded. The population had a slight majority of females (M/F ratio = 0.76), mean age was 47 years. 25% presented infectious uveitis, primarily due to herpetic etiology, toxoplasmosis, and tuberculosis. Non-infectious uveitis was the most prevalent diagnosis (38%), with sarcoidosis, HLA-B27-associated uveitis, and Fuch's uveitis as prominent causes. Anatomically, anterior segment was most frequently involved (41%). Significant improvement in visual acuity was observed at follow-up, particularly in patients with infectious uveitis. CONCLUSIONS: Our study sheds light into the epidemiological landscape of uveitis in Northern Italy, reflecting changing patterns due to factors such as migration and changing sexual habits. In particular, higher percentages of syphilis have been observed, compared to other European reports. The distribution of non-infectious uveitis reflects other epidemiological European series. Higher percentages of neoplastic masquerade syndromes support the need of early recognition. Our findings offer precious insights for uveitis epidemiology and daily clinical practice.

The Humira in Ocular Inflammations Taper (HOT) Study.

PURPOSE: To assess factors that impact the risk of relapse in patients with noninfectious uveitis (NIU) who undergo adalimumab tapering after achieving remission. DESIGN: Retrospective study. METHODS: In this multicenter study, patients with NIU were treated with adalimumab and subsequently tapered. Patient demographics, type of NIU, onset and duration of disease, the period of inactivity before tapering adalimumab, and the tapering schedule were collected. The primary outcome measures were independent predictors of the rate of uveitis recurrence after adalimumab tapering. RESULTS: Three hundred twenty-eight patients were included (54.6% female) with a mean age of 34.3 years. The mean time between disease onset and initiation of adalimumab therapy was 35.2 ± 70.1 weeks. Adalimumab tapering was commenced after a mean of 100.8 ± 69.7 weeks of inactivity. Recurrence was observed in 39.6% of patients at a mean of 44.7 ± 61.7 weeks. Patients who experienced recurrence were significantly younger than those without recurrence (mean 29.4 years vs 37.5 years, P = .0005), and the rate of recurrence was significantly higher in younger subjects (hazard ratio [HR] = 0.88 per decade of increasing age, P = .01). The lowest rate of recurrence was among Asian subjects. A faster adalimumab taper was associated with an increased recurrence rate (HR = 1.23 per unit increase in speed, P < .0005). Conversely, a more extended period of remission before tapering was associated with a lower rate of recurrence (HR = 0.97 per 10-weeks longer period of inactivity, P = .04). CONCLUSIONS: When tapering adalimumab, factors that should be considered include patient age, race, and duration of disease remission on adalimumab. A slow tapering schedule is advisable.

Presumed Müller Cell Activation in Multiple Evanescent White Dot Syndrome.

Purpose: The purpose of this study was to investigate the foveal changes occurring in multiple evanescent white dot syndrome (MEWDS) using multimodal imaging techniques with a specific focus on hyper-reflective dots (HRDs). Methods: This was a retro-prospective observational study including 35 eyes with active MEWDS. Structural and en face optical coherence tomography (OCT) was performed, with follow-up visits at 2 weeks, 6 weeks, and 2 months from baseline. HRD percentage area (HRD % area) was calculated in a 600 µm fovea centered circle on en face OCT, after background subtraction and image binarization. HRD % area was compared with 23 fellow control eyes. Longitudinal changes in the HRD % areas were assessed using repeated-measure statistics. Results: HRDs were observed as scattered hyper-reflective spots on the vitreoretinal interface on en face OCT images, colocalizing with HRDs or vertical hyper-reflective lines on structural OCT images. The baseline evaluation showed a significantly higher HRD % area in MEWDS eyes compared to fellow eyes (0.10 ± 0.03 vs. 0.08 ± 0.04, P = 0.01). The HRD % area correlated positively with LogMAR visual acuity and inversely with the duration of symptoms. Longitudinal analysis revealed a significant reduction in the HRD % area over time. There was no significant interaction between the rate of HRD disappearance and clinical or demographic factors at baseline. Conclusions: As HRD potentially represents the end-feet projections of activated Müller cells on the retinal surface, this study supports the involvement of Müller cells in the pathogenesis of the disease. The findings highlight the potential of en face OCT imaging for monitoring the progression of MEWDS.