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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

To describe the development and management of ocular decompression retinopathy after cataract surgery in a patient with chronic severe HLA-B27 associated anterior uveitis. Case report. A 45-year-old woman affected by ankylosing spondylitis and HLA-B27 associated anterior uveitis was referred for left eye pain and inflammation one week after phacoemulsification and intraocular lens implantation. She had a history of anterior uveitis complicated by iris bombé for posterior synechiae and cataract. Intraocular pressure (IOP) prior surgery was 14 mmHg. Clinical examination after cataract surgery showed intense inflammation in the anterior chamber and marked hypotony. Fundus examination revealed the presence of ocular decompression retinopathy. High-dose corticosteroids were supplemented to control the uveitis, with a progressive IOP increase and resolution of retinal hemorrhages over the following months. Ocular decompression retinopathy may present after cataract surgery in patients with complicated HLA-B27 associated anterior uveitis who develop severe post-surgical hypotony and inflammation. Close monitoring of IOP and intraocular inflammation are warranted to prevent severe complications after cataract surgery in these patients. [ABSTRACT FROM AUTHOR]

Background/aims: To report our five-year experience on vitreoretinal lymphoma (VRL) as a single-center tertiary hospital. Methods: The ophthalmic, cytopathology, and onco-hematologic records of patients with VRL consecutively seen from 2014 to 2019 were reviewed. Results: Fifty-nine eyes of 31 patients with large B-cell VRL were included. Eighty-one percent has developed central nervous system lymphoma at the end of follow-up. Several different imaging findings were noted, including vitritis, leopard spot appearance, Bruch's membrane/RPE infiltrations, and ellipsoid zone disruption. A variable combination of MYD88-L265P mutation in the aqueous and/or in the vitreous and positive cytology/histology allowed to reach a definite diagnosis in all the patients. Therapies included intravitreal injections of methotrexate and rituximab, systemic chemotherapy, pan-encephalic radiotherapy, and hematopoietic stem cell transplantation. Conclusion: No definite guidelines exist for VRL management. It is crucial to collect as much data as possible from tertiary referral hospitals, which suitably manage a conspicuous number of VRL patients. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinical course and management of anterior uveitis complicated by ocular hypotony associated with Hodgkin lymphoma.Design: Case report.Methods: Chart and multimodal imaging review, including ultrasound biomicroscopy, widefield fundus pictures, fundus autofluorescence, fluorescein angiography, and indocyanine green angiography.Results: A 44-year-old female with progressive visual deterioration and history of low-grade fever developed bilateral granulomatous anterior uveitis complicated by severe hypotony maculopathy, not improving with systemic and topical steroids. After starting ibopamine 2% eye drops, ocular hypotony progressively resolved with visual recovery. Histologic examination of a biopsied enlarged lymph node of the neck revealed the presence of Hodgkin lymphoma, for which the patient underwent systemic chemotherapy.Conclusion: Severe hypotony maculopathy complicating anterior uveitis can be associated with Hodgkin lymphoma. Topical ipobamine 2% was safe and effective in the treatment of ocular hypotony in this case. [ABSTRACT FROM AUTHOR]

Purpose: To describe the distinguishing features of retinitis-like lesions seen in vitreoretinal lymphoma (VRL) from viral and toxoplasma retinitis.Methods: In this multicenter, retrospective study, we reviewed charts and imaging of consecutive patients with VRL. The associated features and the characteristics of retinitis-like lesions were assessed and compared with those of viral and toxoplasmic retinochoroiditis. Primary outcome measures were the unique features of VRL retinitis-like lesions.Results: Out of 76 eyes of 38 patients with VRL, retinitis-like lesions were identified in 6 eyes and confirmed on OCT. Distinctive features of VRL retinitis-like lesions were massive retinal thickening, associated sub-retinal pigment epithelium infiltrates and partial restoration of retinal layers after specific therapy.Conclusion: VRL can present with retinitis-like lesions that have distinctive OCT features on presentation as well as healing that can help to differentiate them from other lookalike etiologies and can guide further diagnostic and therapeutic interventions. [ABSTRACT FROM AUTHOR]

To identify main qualitative and quantitative changes by spectral-domain optical coherence tomography (SD-OCT) in eyes with vitreoretinal lymphoma (VRL) shifting form active to remission phase, after intravitreal rituximab (IVR).SD-OCT scans retrospectively assessed for: hyperreflective retinal dots (HRD), intraretinal infiltration, subretinal infiltration, outer retina (OR) fuzzy borders, and pigment epithelium detachments (PED).Central macular thickness (CMT) and choroidal thickness (CT) were measured. These features were examined in active and in remission phase.Eighteen eyes of nine patients enrolled. Patients received 5.3 ± 2.1 IVR according to a 2-weekly or monthly schedule. In remission phase, presence of HRD (p = 0.02), intraretinal infiltration (p = 0.02), OR fuzzy borders (p = 0.01) significantly reduced. Treatment frequency did not influence rate of disappearance of these features. CMT (p = 0.04) and CT (P = 0.004) became thinner.Signs referable to lymphoma-induced inflammation (CMT, CT, HRD) and infiltration (intraretinal infiltrates, OR fuzzy borders) decreased in remission phase. Further comparative studies needed to identify the specific role of IVR in inducing these changes. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of acute retinal necrosis (ARN) occurring in the opposite eye to the ophthalmic shingles eruption.Design: Case report.Methods: Clinical examination and multimodal imaging review, including ultra-widefield photography and fluorescein angiography (FA).Results: A 49-years-old man attended the Uveitis Service for concurrent acute visual loss in the right eye and ophthalmic shingles skin eruption of the left ophthalmic trigeminal branch. Fundus examination of the right eye revealed multiple yellowish patchy areas of retinitis in the peripheral retina consistent with ARN. Multimodal imaging and laboratory tests confirmed the diagnosis.Discussion: Herpes Zoster Ophtalmicus (HZO) represents the 10-20% of Herpes Zoster (HZ) cases and ARN is a rare but sight-threatening complication due to the viral widespread along the retina. By definition, the reactivation of Varicella Zoster virus (VZV) has a unilateral clinical expression, due to the centrifugal neural pathway followed by the virus from the neural ganglia, and HZO, as well, results located on the same side of the skin affection. The case presented contradicted this postulate.Conclusion: prompt diagnosis, through fundus examination and FA, and proper antiviral therapy were the mainstays to counteract the posterior uveitis. Nowadays, vaccination is a safe weapon to efficaciously reduce the ZVZ incidence among general population. [ABSTRACT FROM AUTHOR]

Purpose: To describe clinical features and outcome in bilateral acute retinal necrosis (BARN). Methods: Observational retrospective longitudinal review of ocular findings. Results: Thirty eyes of 15 patients (age 44.1 ± 15.8). Delay of involvement between eyes was 57.2 ± 105.2 months (median 3, range 0.5-360). Herpes simplex virus (HSV)-1 was the most frequent (20 eyes, 66.6%), followed by HSV-2 (five eyes, 16.7%) and varicella zoster virus (VZV, four eyes, 13.3%). Visual acuity worsened in 7 (23%) eyes, improved in 4 (13%), and remained stable in 19 (63%). Major complications included retinal detachment (11 eyes, 36%), optic atrophy (11 eyes, 33%), proliferative vitreoretinopathy (four eyes, 13.3%), neovascular glaucoma (four eyes, 13.3%), phthisis bulbi (three eyes, 10%). Symptoms-to-referral average time was 2.7 ± 1.0 weeks (range 1-4). Conclusions: In our study BARN was associated with severe visual outcome and high rate of ocular complications. Although BARN is a rare disease, the course is aggressive, regardless prompt referral in tertiary-care uveitis centers. [ABSTRACT FROM AUTHOR]

Multiple myeloma (MM) therapy is evolving, and several new drugs are now available, extending patients' life and exposure to different compounds and toxicities. We conducted a cross-sectional observational study enrolling 93 consecutive patients on active treatment for MM, aiming to assess their ocular complications. All the patients underwent a comprehensive ophthalmic evaluation. In our cohort, prevalence of low visual acuity was in line with similar age healthy population reported in registry studies. Interestingly, we recorded a higher prevalence of lens opacities (46%) and dry eye syndrome (53%). Nevertheless, we did not find any significant association between ocular disorders and anti-myeloma treatments, even steroid therapy. This observation suggests that other factors besides treatments, such as M-protein deposition in eye structures, may have a role in developing ocular toxicities. Since MM patients are elderly patients at higher risk of age-related eye disorders, we recommend periodic ophthalmic assessment in daily practice. [ABSTRACT FROM AUTHOR]

Italy was the first European country to be affected by the SARS-CoV-2 pandemic. In this scenario, we had to face a new clinical approach in our Pediatric Rheumatology Unit for the management of patients affected by juvenile idiopathic arthritis (JIA)-associated uveitis. During the lockdown (phase 1), the weekly outpatient clinic was discontinued and telephone consultations were set up. A toll-free telephone number was instituted for emergencies. None of our children with JIA-associated uveitis was advised to stop the ongoing immunosuppressant systemic therapy. We had no cases of COVID-19 infection and uveitis activity was under control in all but two out of 125 patients, which was comparable with the pre-COVID-19 situation. During phase 2 of the pandemic, hospital and ambulatory rearrangements were made to minimize the risk of SARS-CoV-2 infection. Overall, during the first 4 weeks of phase 2, we did not notice an increased number of patients with uveitis activity. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the long-term efficacy of golimumab in patients with severe recalcitrant uveitis who had inadequate response to previous biologics. Methods: Retrospective study (13 patients with JIA, 4 with HLA-B27-associated uveitis). Indication for treatment was active uveitis despite biologics. Golimumab dosing was 50 mg monthly/subcutaneously. Main outcome measures: uveitis activity, visual acuity improvement, reduction of systemic therapy (corticosteroids/immunosuppressants), adverse events. Results: Of 17 patients (34 affected eyes), response to golimumab was seen in 14 patients; at last visit uveitis was inactive in 12 patients. Three patients were nonresponders. Mean follow-up time on golimumab was 21.9 months. Visual acuity remained stable in 26 eyes, improved in 7, and worsened in 1. Mean systemic prednisolone dose before and after golimumab was 12.5-3.5 mg/day. One patient developed pulmonary infection. Conclusions: Golimumab may be a promising new therapeutic option for severe uveitis patients who have not responded to other biologics. [ABSTRACT FROM AUTHOR]

Objective: To assess health-related quality of life with Italian SF-36 in patients with uveitis on chronic systemic immunosuppressants. Methods: Consecutive patients with chronic noninfectious uveitis on systemic immunosuppressive treatment seen at the Ocular Immunology Service, San Raffaele Scientific Institute, Milan, were enrolled. Health status was evaluated with the Italian version of the SF-36 questionnaire in 8 dimensions: physical functioning, physical disability, body pain, general health, vitality, social functioning, emotional disability, mental health. Statistical analysis of variance was used to test for differences in SF-36 scores between subgroups of interest (age, sex, educational level, location and laterality of uveitis, systemic associated diseases, visual acuity, systemic treatment, duration of disease). Results: One hundred Caucasian patients (35 female, 65 male) with a median age of 46.31 years (range 10–76) were examined. Uveitis was bilateral in 88% of patients. Uveitis location was anterior (12%), intermediate (10%), posterior (34%), and panuveitis (44%). The most frequent diagnoses were idiopathic (32%), sarcoidosis (15%), and Behçet disease (15%). The SF-36 physical and mental component summary scores were lower among uveitis patients than those of the general Italian population; the most statistically significant differences found in subgroups were older age, female sex, low visual acuity (<20/40), and long-standing duration of the disease (>6 years). Conclusions: Italian patients with uveitis on chronic systemic immunosuppressants reported markedly poorer general health status than normal matched population subjects. The quality of life of these patients seems to be significantly related to visual acuity and disease duration. [ABSTRACT FROM AUTHOR]

Lymphomas are the most common orbital malignancies, marginal zone B-cell lymphoma being the most common histotype. One quarter of these lymphomas exhibit conjunctival involvement, which is almost exclusively observed in indolent lymphomas (96% of conjunctival lymphomas). Primary conjunctival marginal zone B-cell lymphoma exhibits interesting and peculiar features, with some molecular, biological, clinical and prognostic differences from the rest of the ocular adnexal lymphomas. Recent studies demonstrate that chromosomal abnormalities, autoimmune disorders and infectious agents are variably associated with the development and behavior of this lymphoma. Conjunctival lymphomas are rarely lethal tumors and, in selected cases, may be managed without anti-tumor treatment. In other cases, conjunctival lymphomas cause symptoms that require immediate treatment. To date, no guidelines for the management of patients with conjunctival lymphoma exist. Surgical resection, radiotherapy and chemotherapy are the most common approaches to these malignancies, but the use of systemic immunotherapy, bacteriaeradicating antibiotic therapy and local treatments, such as intralesional injections of interferon or rituximab and eyedrops of cytostatics, is progressively growing. [ABSTRACT FROM AUTHOR]

We present a case of a patient with bilateral posterior uveitis HLA-A29 positive, masquerading intraocular lymphoma. A 43 year-old woman presented with bilateral vitritis and chorioretinal lesions compatible with 'birdshot lesions'. The patient was initially diagnosed with birdshot retinochoroidopathy and later on, during follow up, the occurrence of neurologic involvement and the lack of response to systemic immunosuppression led us to re-evaluate the diagnosis. A definite diagnosis of intraocular lymphoma with central nervous system involvement was made. This case is presented to highlight the importance of careful follow-up of patients with chronic uveitis and re-evaluation of systemic symptoms and signs, in particular when ocular findings are highly suggestive for masquerade syndrome. [ABSTRACT FROM AUTHOR]

Zoledronic acid is the most effective treatment in patients with hypercalcemia secondary to malignancy; occasionally it can be related to ocular inflammation. We report a case of severe unilateral anterior uveitis following administration of intravenous zoledronic acid in a patient with frontal hyperostosis secondary to breast cancer. The patient developed anterior uveitis 24 hours after the administration of intravenous zoledronate. The patient was treated with topical prednisone and cycloplegic eye drops, with complete recovery after one month. This report shows that, on rare occasions, zoledronic acid can be a possible cause of acute anterior uveitis. [ABSTRACT FROM AUTHOR]