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1.
Br J Ophthalmol ; 2024 May 29.
Article in English | MEDLINE | ID: mdl-38811053

ABSTRACT

BACKGROUND: The aim of the Posner-Schlossman Syndrome European Study Group (PSS-ESG) is to acquire a comprehensive dataset of European patients with PSS. Here, we present the first report on the study protocol and the clinical findings of the patients at baseline. METHODS: The PSS-ESG is a retrospective, multicentre study designed to evaluate patients with PSS. The study, designed and driven by a European Expert Committee includes three datasets: (1) the baseline, (2) the follow-up and (3) the intraocular pressure (IOP)/glaucoma dataset. RESULTS: A total of 11 centres adhered to the PSS-ESG and 107 patients were included (68 males, 39 females) mostly Caucasian (93.4%). At uveitis onset, the patient's age ranged between 11 and 76 years, (mean age: 42±15 years).Best-corrected visual acuity was >0.5 in 80.3% of the eyes, IOP was >40 mm Hg in 44% of the eyes. Keratic precipitates were found in 78.5% of the eyes. No flare or cells in anterior chamber were detected in 56% and 53% of the cases, respectively. PCR analysis on aqueous sample was positive for cytomegalovirus-DNA in 50.6% out of the 81 tested patients. CONCLUSIONS: The PSS-ESG is the first multicentre study aimed to collect a comprehensive dataset of patients with PSS in non-Asian countries. A middlde-aged Caucasian male with a low-grade anterior chamber inflammation, keratic precipitates, preserved visual acuity and marked increased in IOP seemed to be the standard PSS patient across the 11 uveitis and glaucoma centres participating in the PSS-ESG.

2.
J Ophthalmic Inflamm Infect ; 14(1): 22, 2024 May 30.
Article in English | MEDLINE | ID: mdl-38814386

ABSTRACT

BACKGROUND: Non-infectious uveitis affecting the posterior segment of the eye (NIU-PS) is an inflammatory disease, which can significantly impair visual acuity if not adequately treated. Fluocinolone-acetonide sustained-release-0.2 µg/day intravitreal (FAc) implants are indicated for prevention of relapse in recurrent NIU-PS. The aim here was to provide treating clinicians with some consensus-based-recommendations for the clinical management of patients with NIU-PS with 0.2 µg/day FAc implants. METHODS: A European-clinical-expert-group agreed to develop a consensus report on different issues related to the use of FAc implants in patients with NIU-PS. RESULTS: The Clinical-expert-panel provided specific recommendations focusing on clinical presentation (unilateral/bilateral) of the NIU-PS; systemic involvement of NIU-PS and the lens status. Treatment algorithms were developed; one that refers to the management of patients with NIU-PS in clinical practice and another that establishes the best clinical scenarios for the use of FAc implants, both as monotherapy and as adjuvant therapy. Additionally, the Clinical-expert-panel has provided recommendations about the use of the FAc implants in a clinical-setting. The Clinical-expert-panel also considered the safety profile of FAc implants and their possible implications in the daily practice. CONCLUSIONS: As more clinical experience has been gained using FAc implants, it was necessary to update the clinical recommendations that guide patient management in the clinic. The current consensus document addresses relevant issues related to the use of FAc implants on different types of patients with various etiologies of NIU-PS, and was conducted to standardize approaches to help specialists obtain better clinical outcomes.

3.
Ocul Immunol Inflamm ; : 1-6, 2024 May 22.
Article in English | MEDLINE | ID: mdl-38776460

ABSTRACT

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.

4.
Am J Ophthalmol Case Rep ; 34: 102046, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38633003

ABSTRACT

Purpose: To describe a case of bilateral panuveitis in a patient with Stimulator of Interferon Genes (STING)-Associated Vasculitis with Onset in Infancy (SAVI). Observations: A 45-year-old patient diagnosed with SAVI presented bilateral panuveitis and uncontrolled secondary intraocular hypertension due to structural complications from uveitis. Multimodal imaging revealed the presence of intraretinal fluid and bilateral vasculitis. The patient was started with systemic methotrexate. Conclusions and importance: This case is essential to characterize ocular involvement in patients with SAVI. Awareness of these ocular manifestations is crucial for timely management and improvement of visual prognosis.

5.
Br J Ophthalmol ; 2024 Apr 04.
Article in English | MEDLINE | ID: mdl-38575198

ABSTRACT

BACKGROUND: Juvenile idiopathic arthritis (JIA)-associated uveitis typically presents as a silent chronic anterior uveitis and can lead to blindness. Adherence to current screening guidelines is hampered by complex protocols which rely on the knowledge of specific JIA characteristics. The Multinational Interdisciplinary Working Group for Uveitis in Childhood identified the need to simplify screening to enable local eye care professionals (ECPs), who carry the main burden, to screen children with JIA appropriately and with confidence. METHODS: A consensus meeting took place in January 2023 in Barcelona, Spain, with an expert panel of 10 paediatric rheumatologists and 5 ophthalmologists with expertise in paediatric uveitis. A summary of the current evidence for JIA screening was presented. A nominal group technique was used to reach consensus. RESULTS: The need for a practical but safe approach that allows early uveitis detection was identified by the panel. Three screening recommendations were proposed and approved by the voting members. They represent a standardised approach to JIA screening taking into account the patient's age at the onset of JIA to determine the screening interval until adulthood. CONCLUSION: By removing the need for the knowledge of JIA categories, antinuclear antibody positivity or treatment status, the recommendations can be more easily implemented by local ECP, where limited information is available. It would improve the standard of care on the local level significantly. The proposed protocol is less tailored to the individual than the 'gold standard' ones it references and does not aim to substitute those where they are being used with confidence.

6.
Invest Ophthalmol Vis Sci ; 65(4): 28, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-38630674

ABSTRACT

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.


Subject(s)
Retina , White Dot Syndromes , Humans , Visual Acuity , Retina/physiology , Fovea Centralis , Tomography, Optical Coherence
7.
Clin Exp Rheumatol ; 42(5): 974-982, 2024 May.
Article in English | MEDLINE | ID: mdl-38530666

ABSTRACT

OBJECTIVES: This study aims to evaluate the efficacy and safety of JAK inhibitors (JAKi) in a monocentric cohort of adult patients with juvenile idiopathic arthritis (JIA). METHODS: Patients attending a rheumatology transition clinic were retrospectively included in case of: i) JIA diagnosis according to current classification criteria (1); ii) age ≥18 years and iii) treatment with JAKi for at least 3 months. RESULTS: Seventeen adult patients with JIA were treated with JAKi (as first JAKi, 9 patients (52.9%) received tofacitinib and 8 (47.1%) baricitinib). At 3 months after JAKi initiation, 8 patients (47%) achieved a response and 4 patients (23.5%) achieved disease remission (3 patients with baricitinib and 1 with tofacitinib, 37.5% vs. 16.7%, p=0.294). None of those with systemic JIA and enthesitis-related arthritis obtained remission; the remission rate at 3 months was higher, although not significantly, in the oligoarticular subset compared to the polyarticular subset (37.5% vs. 20%). Patients with ≤1 active joint involvement at JAKi start had a higher remission rate (50% vs. 22.2%). Subjects who achieved remission on JAKi had a significantly lower pre-treatment DAS28-CRP compared to those with still active disease (p=0.010, Mann-Whitney U=4). A pre-treatment DAS28-CRP <3.76 predicted response to JAKi with 100% sensitivity and 84.6% specificity (p=0.023). The remission rate was lower among patients who had been treated with ≥2 biological drugs before JAKi start (9% vs. 66.7%; p=0.05). One patient in concomitant treatment with leflunomide developed severe arterial hypertension. CONCLUSIONS: JAKi may represent an effective and safe treatment option for adult JIA patients with low/moderate disease activity, particularly in case of oligoarticular involvement.


Subject(s)
Arthritis, Juvenile , Azetidines , Janus Kinase Inhibitors , Piperidines , Purines , Pyrazoles , Pyrimidines , Remission Induction , Sulfonamides , Humans , Arthritis, Juvenile/drug therapy , Retrospective Studies , Janus Kinase Inhibitors/therapeutic use , Janus Kinase Inhibitors/adverse effects , Male , Female , Adult , Piperidines/therapeutic use , Piperidines/adverse effects , Pyrimidines/therapeutic use , Pyrimidines/adverse effects , Treatment Outcome , Pyrazoles/therapeutic use , Pyrazoles/adverse effects , Azetidines/therapeutic use , Azetidines/adverse effects , Young Adult , Sulfonamides/therapeutic use , Adolescent , Purines/therapeutic use , Purines/adverse effects , Antirheumatic Agents/therapeutic use , Antirheumatic Agents/adverse effects , Time Factors
8.
Ocul Immunol Inflamm ; : 1-7, 2024 Mar 04.
Article in English | MEDLINE | ID: mdl-38436928

ABSTRACT

PURPOSE: Vitreoretinal lymphoma (VRL) is a rare lymphoma affecting the vitreous and the retina. Clinical diagnosis is challenging and often delayed and may lead to aggravated prognosis. This study aims to review multimodal imaging findings in VRL. METHODS: We performed a comprehensive narrative review of the multimodal imaging findings that might be useful in the detection of VRL lesions. RESULTS: The most frequent ocular manifestations of VRL are vitritis, and retinal and sub-retinal Pigmented Epithelium (RPE) infiltrations. Color Fundus Photography (CFP) detects vitreous haze, optic nerve, retinal and sub-RPE infiltration. Ultra-wide field imaging allows visualization of different patterns of vitreous haze and monitoring of VRL evolution through the detection of chorio-retinal atrophy (CRA). Fundus Autofluorescence shows granular hypo- and hyper-autofluorescent pattern. Optical Coherence Tomography (OCT) reveals vitreous cells, vertical hyper-reflective lesions and sub-RPE infiltrates. Fluorescein Angiography (FA) shows hypo or hyperfluorescent round lesions at the late stages of the examination, while Indocyanine Green Angiography (ICGA) detects round areas of focal hypo-fluorescence in the early phases that gradually enlarge in the late phases. B-scan ultrasonography detects vitreous opacities and homogeneous hyperreflective corpuscular material in the vitreous, and is a strongly recommended tool in suspecting VRL and is particularly useful when vitreous haze is impeding retinal examination. CONCLUSION: Diagnostic vitrectomy with cytopathological analysis remains the gold standard for VRL diagnosis, however multimodal imaging allows the identification of suggestive retinal and vitreal lesions for early suspicion, diagnosis, and treatment and monitoring disease progression and response to treatment.

9.
Article in English | MEDLINE | ID: mdl-38331409

ABSTRACT

OBJECTIVES: To investigate clinical features associated with lack of response to MTX in juvenile idiopathic arthritis associated uveitis (JIA-U). METHODS: Clinical records of JIA-U patients were retrospectively reviewed. Differences among variables were assessed by Mann-Whitney and χ 2 or Fisher's exact tests as appropriate. Association between predictors and requirement of a biological disease modifying antirheumatic drug (bDMARD) was evaluated by univariate Cox regression analysis and Kaplan-Meier curves. A multivariable logistic model was applied to estimate strength of association, adjusting for potential confounders. RESULTS: Data from 99 JIA-U patients treated with MTX were analysed (82.8% female), with a mean follow up of 9.2 years and a mean age at uveitis onset of 5.7 years. In 65 patients (65.7%) at least one bDMARD to control uveitis was required. Children requiring a bDMARD for uveitis had lower age at JIA and uveitis onset, more frequent polyarticular course, higher frequency of bilateral uveitis at onset and higher prevalence of systemic steroids' use. Despite similar frequency of ocular damage at onset, MTX non responders showed a higher percentage of ocular damage at last visit. Younger age at JIA onset, polyarticular course and a history of systemic steroids' use resulted independent factors associated to lack of response to MTX at Cox regression analysis. Kaplan-Meier curves and the multivariate model confirms the independent role of both polyarticular course and systemic steroids' use. CONCLUSIONS: Younger age at JIA onset, polyarticular course and a history of systemic steroids' use are predictors of a worse response to MTX in JIA-U.

10.
Ocul Immunol Inflamm ; 32(3): 355-357, 2024 Apr.
Article in English | MEDLINE | ID: mdl-36696367

ABSTRACT

INTRODUCTION: Endogenous endophthalmitis (EE) is a severe intraocular infection due to hematogenous spread of bacteria from an extraocular site. Recognition of the primary source of hematogenous spread of bacteria is essential to establish an adequate therapy and avoid other major complications. Infected cutaneous tumor has never been reported as a possible source of EE. PURPOSE: To describe the first case of EE due to hematogenous spread of methicillin-sensitive Staphylococcus aureus from an infected cutaneous basal cell carcinoma. Systemic antibiotic therapy and surgical excision of the cutaneous lesion were performed. CONCLUSION: Severe and long-standing skin infections should be considered as a rare cause of EE.


Subject(s)
Carcinoma, Basal Cell , Endophthalmitis , Skin Neoplasms , Staphylococcal Infections , Humans , Endophthalmitis/diagnosis , Endophthalmitis/drug therapy , Endophthalmitis/etiology , Anti-Bacterial Agents/therapeutic use , Staphylococcal Infections/complications , Staphylococcal Infections/diagnosis , Staphylococcal Infections/drug therapy , Bacteria , Skin Neoplasms/complications , Skin Neoplasms/diagnosis , Skin Neoplasms/drug therapy , Carcinoma, Basal Cell/complications , Carcinoma, Basal Cell/diagnosis , Carcinoma, Basal Cell/drug therapy
11.
Eye (Lond) ; 38(5): 910-916, 2024 Apr.
Article in English | MEDLINE | ID: mdl-37904001

ABSTRACT

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.


Subject(s)
Macular Edema , Uveitis , Humans , Macular Edema/drug therapy , Fluorescein Angiography , Uveitis/drug therapy , Biomarkers , Tomography, Optical Coherence/methods , Intravitreal Injections , Dexamethasone , Retrospective Studies , Treatment Outcome
12.
Eur J Ophthalmol ; 34(2): NP108-NP112, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37817537

ABSTRACT

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.


Subject(s)
Cytomegalovirus Retinitis , Endophthalmitis , Retinitis , Syphilis , Uveitis , Humans , Male , Middle Aged , Syphilis/diagnosis , Syphilis/drug therapy , Uveitis/diagnosis , Uveitis/drug therapy , Retinitis/diagnosis , Retinitis/drug therapy , Cytomegalovirus Retinitis/diagnosis , Cytomegalovirus Retinitis/drug therapy , Inflammation
13.
Am J Ophthalmol ; 258: 87-98, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37734639

ABSTRACT

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.


Subject(s)
Inflammation , Uveitis , Humans , Female , Adult , Male , Adalimumab/therapeutic use , Retrospective Studies , Uveitis/diagnosis , Uveitis/drug therapy , Recurrence , Vision Disorders , Treatment Outcome
14.
Br J Ophthalmol ; 2024 Jan 17.
Article in English | MEDLINE | ID: mdl-37963712

ABSTRACT

BACKGROUND: Intermediate and posterior manifestations of vitreoretinal lymphoma (VRL) are well characterised. However, there is limited information on anterior segment involvement in VRL. This study aimed to describe the anterior manifestations of VRL, and their association with molecular testing. METHODS: Retrospective analysis of patients with biopsy-proven VRL. Study variables included anterior segment manifestations, findings from slit-lamp photos and in vivo confocal microscopy (IVCM) when available. MYD88 L265P mutation and cytology in the aqueous humour, retinal and systemic findings were also analysed. RESULTS: The analysis included 108 eyes of 55 VRL patients. Anterior segment involvement was present in at least one visit in 55 eyes (51%) of 33 patients (60%); it included keratic precipitates (dendritiform with branching and irregular margins in 33 eyes, dust-like in 16 eyes and large granulomatous in 12 eyes), cells in the anterior chamber (51 eyes) and posterior synechiae (2 eyes). IVCM was available for 41 eyes and showed different morphologies of keratic precipitates, including floral, spikes and mulberry patterns (66%, 56% and 20%, respectively). MYD88 L265P mutation in the aqueous humour was detected in 10/21 (48%) eyes with no anterior segment involvement and 24/37 (65%) eyes with anterior segment involvement. CONCLUSIONS: Anterior segment manifestations are often present in VRL and include dendritiform and dust-like keratic precipitates. IVCM in VRL can identify different patterns associated with keratic precipitates. MYD88 L265P mutation in the aqueous humour of VRL patients can also be found in eyes without significant anterior segment involvement.

15.
Eur J Ophthalmol ; : 11206721231211931, 2023 Nov 13.
Article in English | MEDLINE | ID: mdl-37956540

ABSTRACT

Vitreoretinal lymphoma (VRL) is a rare B-cell intraocular neoplasia characterized by poor long-term prognosis and lack of effective therapies. It mainly involves the vitreous humor, the retina, and the retinal pigment epithelium (RPE), although anterior segment involvement can occur. VRL is classified as a lymphoma of immune privileged sites, along with testis lymphoma and primary central nervous system lymphoma (PCNSL). VRL and PCNSL are strictly connected indeed: 80% of VRL develop PCNSL, while 20% of patients with PCNSL present VRL during natural history of lymphoma. Due to the lack of worldwide consensus about diagnosis, therapy, and follow-up timing, VRL represents one of the most challenging ocular affections.VRL commonly masquerades as a posterior uveitis, and misdiagnosis often occurs because of partial response to topical steroids. Gold standard for diagnosis is cytological analysis of vitreous humor. However, this technique lacks sensitivity and supplemental molecular analyses can improve the diagnostic process. Multimodal imaging allows ophthalmologists to empower their clinical suspicion and a comprehensive examination can highlight typical features of VRL and justify further invasive procedures.There is no consensus about VRL therapy, and none of the therapeutical scheme has demonstrated to prevent cerebral involvement and improve patient's overall survival. Intravitreal injections of chemotherapeutics drugs, ocular radiation therapy and systemic chemotherapy can be considered in the treatment of VRL. Once cerebral involvement occurs, systemic chemotherapy must be included in the treatment as a life-saving therapy. Further multicentric studies are required to find out the best treatment of patients with VRL.

16.
Invest Ophthalmol Vis Sci ; 64(13): 20, 2023 10 03.
Article in English | MEDLINE | ID: mdl-37824135

ABSTRACT

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.


Subject(s)
Retinal Diseases , White Dot Syndromes , Humans , Retinal Diseases/diagnosis , Ependymoglial Cells , Fluorescein Angiography/methods , White Dot Syndromes/diagnosis , Retina , Tomography, Optical Coherence/methods , Retrospective Studies
17.
Retina ; 43(11): 1960-1970, 2023 11 01.
Article in English | MEDLINE | ID: mdl-37657077

ABSTRACT

PURPOSE: To perform an unsupervised machine learning clustering of patients with punctate inner choroidopathy (PIC) and provide new insights into the significance of pachychoroid disease features in PIC eyes. METHODS: Retrospective multicenter study, including 102 eyes from 82 patients diagnosed with PIC. Demographics, clinical data, and multimodal imaging, including fundus photography, optical coherence tomography, and indocyanine green angiography, were collected. Clusters of eyes were identified, and multilevel logistic regression analysis was performed to compare between-group differences. RESULTS: Using 17 clinical features, two distinct clusters of patients with PIC were identified. Cluster 1 patients were characterized by older age, high myopia, myopic maculopathy features, thin choroids, multiple lesions, and a higher likelihood of developing patchy chorioretinal atrophy. Cluster 2 consisted of younger age, emmetropia or low myopia, thick choroids, choroidal vascular hyperpermeability on late-phase indocyanine green angiography, and high prevalence of focal choroidal excavation. These features exhibited significant differences ( P < 0.05) between the two clusters. CONCLUSION: While PIC typically affects young myopic female patients with thin choroids, a subset of patients with PIC exhibits features associated with pachychoroid disease. Considering the potential influence of choroidal venous insufficiency on PIC manifestations and secondary complications, we propose the term "punctate inner pachychoroidopathy" to characterize this distinct subtype of PIC.


Subject(s)
Myopia , White Dot Syndromes , Female , Humans , Choroid/pathology , Demography , Fluorescein Angiography/methods , Indocyanine Green , Inflammation , Myopia/pathology , Retrospective Studies , Tomography, Optical Coherence/methods , White Dot Syndromes/diagnosis , Male
18.
Ocul Immunol Inflamm ; : 1-9, 2023 Sep 15.
Article in English | MEDLINE | ID: mdl-37712945

ABSTRACT

PURPOSE: To report the clinical and multimodal imaging features of sympathetic ophthalmia in the acute and chronic phases. METHODS: Retrospective cohort study of consecutive patients with sympathetic ophthalmia seen at a tertiary referral center. Charts, imaging studies, and histopathological specimens were reviewed. The clinical features and multimodal imaging in the sympathizing eye were analyzed by sorting features into those seen in the acute and chronic phase. RESULTS: Ten patients were included in the analysis and all of them had previous ocular trauma or complicated retinal detachment. In the acute phase, 70% had anterior uveitis, 70% had vitritis, and 100% had active posterior uveitis; posterior uveitis included multifocal choroiditis (80%), optic disc swelling (40%), multiple serous retinal detachments (20%), MEWDS-like findings (10%), and retinal vasculitis with chorioretinitis (10%). In the chronic phase, posterior manifestations included widespread patches of chorioretinal atrophy in the mid- and far-periphery (80%), peripapillary subretinal fibrosis (50%), and nummular perivascular atrophy (50%). CONCLUSIONS: Sympathetic ophthalmia shows different posterior segment manifestations in the acute and chronic phase. Active sympathetic ophthalmia should be ruled out in eyes with a MEWDS-like presentation or rapidly progressing chorioretinitis, and history of trauma in the fellow eye. Peripapillary subretinal fibrosis and perivascular nummular atrophy may be useful features to suspect SO once acute inflammation has resolved.

19.
Retin Cases Brief Rep ; 17(5): 528-532, 2023 Sep 01.
Article in English | MEDLINE | ID: mdl-37643037

ABSTRACT

PURPOSE: To report a case of fungal endogenous endophthalmitis from Cladosporium sphaerospermum in a patient with juvenile idiopathic arthritis receiving chronic immunosuppressive therapy with golimumab (tumor necrosis factor-α blocker). METHODS: Case report and review of the literature. RESULTS: A 34-year-old woman receiving chronic immunosuppressive therapy with golimumab for juvenile idiopathic arthritis was referred for unilateral visual loss and ocular pain. Worsening conditions after corticosteroid therapy and raised serum beta-D-glucan levels pointed to an infectious fungal etiology. Panfungal polymerase chain reaction-based genetic sequencing on vitreous specimens obtained during vitrectomy detected C. sphaerospermum. The patient management combined surgical treatment and systemic and intravitreal voriconazole. CONCLUSION: Endogenous fungal endophthalmitis can be a rare complication in patients undergoing chronic immunosuppressive therapy (including golimumab) without other predisposing factors. Prompt diagnosis and appropriate treatment are the keys to preserve vision.


Subject(s)
Arthritis, Juvenile , Endophthalmitis , Eye Infections, Fungal , Female , Humans , Adult , Antibodies, Monoclonal , Endophthalmitis/diagnosis , Endophthalmitis/etiology , Eye Infections, Fungal/diagnosis
20.
Prog Retin Eye Res ; 97: 101207, 2023 11.
Article in English | MEDLINE | ID: mdl-37574123

ABSTRACT

White spot syndromes (WSS) pose challenges in the field of ophthalmology, particularly in terms of accurate diagnosis and effective management. However, recent advancements in multimodal imaging (MMI) have significantly contributed to our understanding of WSS, allowing for improved characterization of these inflammatory chorioretinopathies. By employing various imaging modalities, including fundus fluorescein angiography, indocyanine green angiography, fundus autofluorescence, optical coherence tomography (OCT), ultra-widefield imaging, and OCT angiography, researchers and clinicians have gained valuable insights into the underlying pathophysiological changes and clinical progression of WSS. Furthermore, MMI has unveiled novel and atypical variants within the spectrum of WSS, expanding our knowledge in this field. Notably, the identification of secondary forms of WSS occurring concurrently with unrelated chorioretinal disorders has suggested a potential autoimmune mechanism underlying these conditions. The introduction of MMI has also facilitated a more comprehensive evaluation of previously ill-defined entities, such as acute zonal occult outer retinopathy, leading to improved diagnostic criteria and enhanced recognition of distinct features. This review paper provides a comprehensive overview of the latest advances and interpretations in WSS. By integrating MMI into the diagnosis and management of these conditions, this review aims to enhance patient outcomes and provide valuable insights into the complexities surrounding WSS.


Subject(s)
Choroid Diseases , Retinal Diseases , White Dot Syndromes , Humans , Retrospective Studies , White Dot Syndromes/diagnosis , Retinal Diseases/diagnosis , Multimodal Imaging/methods , Fluorescein Angiography/methods , Tomography, Optical Coherence/methods
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