Unusual Presentation of Unilateral Choroidal Melanoma with Bilateral Vasculitis in Young Individual: A Case Report and Review of Literature.

Ocular melanoma stands as the predominant primary intraocular malignancy, albeit infrequently exhibiting ipsilateral inflammatory manifestations. In this article, we present an exceptional case involving a middle-aged male who presented with unilateral ocular choroidal melanoma alongside bilateral retinal vasculitis. The patient initially received temporary steroid treatment, followed by brachytherapy, which contributed to the resolution of vasculitis symptoms. The study aims to document the atypical occurrence of bilateral retinal vasculitis, which could potentially masquerade as melanoma, emphasizing the need for heightened vigilance and further investigations when encountering choroidal masses in its presence. Future research endeavors are warranted to better understand the incidence of such occurrences in this context.

Autoimmune Liver Disease Associated Uveitis: An Extrahepatic Manifestation or a Polyautoimmunity Phenomenon? Case Reports.

PURPOSE: To report two cases of non-granulomatous unilateral anterior uveitis in two female patients associated with autoimmune liver diseases (ALD), emphasizing the possibility of this rare coexistence as a polyautoimmunity phenomenon. CASE DESCRIPTIONS: Case 1: An 18-year-old female with a history of congenital renal hypoplasia and metabolic syndrome presented with anterior uveitis in OS and a history of jaundice, blood elevated hepatic enzymes, and cholangioresonance compatible with primary sclerosing cholangitis (PSC). Laboratory work-up for additional autoimmune and infective causes were within normal limits. Case 2: An 58-year-old female presented an episode of anterior uveitis in OD and a history of Sjögren syndrome diagnosed at the age of 53, primary biliary cholangitis (PBC), systemic sclerosis, Raynaud's phenomenon, bilateral sacroiliitis, and vitiligo, consistent with polyautoimmunity and multiple autoimmune syndrome. CONCLUSIONS: Uveitis rarely coexists with ALD. However, it is essential to recognize the possibility of polyautoimmunity in patients presenting with ophthalmic manifestations and a previous diagnosis of ALD, such as PSC or PBC.

The Role of Complement System's C3 and C4 Fractions in the Pathogenesis of Uveitis.

PURPOSE: To study the role of the complement system's C3 and C4 fractions in the pathogenesis of different types of uveitis. METHODS: A prospective case-control study. 118 patients were enrolled. The control group comprised 60 patients who were otherwise healthy people undergoing cataract or pterygium surgery, whereas the uveitis patients group consisted of 58 people. The levels of C3 and C4 fractions in the blood and in the aqueous humor for both groups were evaluated and compared. RESULTS: No statistically significant differences were found in the levels of the C3 and C4 fractions in the blood between the groups. However, a statistically significant difference was observed in the levels of C3 and C4 in the aqueous humor between the case and control groups, as C3 and C4 fractions were not detected in the control group. The analysis of the mean gradient between the C4 levels in the blood samples and in the aqueous samples did not reveal a statistically significant difference between the case and control groups. However, upon performing an analogous mean gradient analysis of C3 levels, a statistically significant elevation in the value of the mean gradient was observed in the case group as compared to the control group. CONCLUSION: Our findings are in line with our initial hypothesis, that the complement system's C3 and C4 fractions may have a role in the pathogenesis of uveitis.

Uveitis-Glaucoma-Hyphema Syndrome Secondary to Implantable Collamer Lens.

Purpose: To report the first case of uveitis-glaucoma-hyphema (UGH) syndrome post implantable collamer lens (ICL).Methods: Case reportResults: A 41-year-old female presented to our clinic complaining of bilateral eye pain and redness for two weeks. Her past medical history was significant for ICL, in both eyes and multiple sclerosis controlled with treatment. She had a long-standing history of bilateral recurrent uveitis and glaucoma. Ultrasound biomicroscopy revealed several sulcus cysts displacing the ICLs haptic into the ciliary body, leading to iris abrasion and uveitis-glaucoma-hyphema syndrome.Conclusion: We present the first published case worldwide about UGH syndrome secondary to ICL. This is an unusual complication, and measures can be taken to avoid it. This provides evidence of the importance of postoperative follow-up by the surgeon and appropriate work-up when such cases are suspected.

Mimickers of anterior uveitis, scleritis and misdiagnoses- tips and tricks for the cornea specialist.

BACKGROUND: Anterior uveitis, inflammation of the anterior chamber and related structures, is a cohort of diseases that can present to almost any general or sub-specialty Ophthalmology practice. Its features classically involve anterior chamber cell and flare. Below the surface of these two signs exist a panoply of diagnoses. BODY: The purpose of this review is to provide a general framework for diagnoses of anterior uveitis that are often missed as well as non-uveitic pathologies that often mimic anterior uveitis. Diagnostic deviation in either direction can have vision-threatening and rarely life-threatening consequences for patients. Using a comprehensive literature review we have collected a broad spectrum of etiologies of anterior uveitis that are easily missed and non-uveitic pathologies that can masquerade as anterior uveitis. CONCLUSIONS: We present a focused review on specific misdiagnosed anterior uveitis pathologies and some of the conditions that can masquerade as anterior uveitis and scleritis.

Tubulointerstitial Nephritis and Uveitis: A Case Report.

Tubulointerstitial nephritis and uveitis (TINU) is a rare disease of unknown pathogenesis that is characterized by tubulointerstitial nephritis and uveitis. Currently, there are over 250 reported cases of TINU syndrome. TINU syndrome typically presents with mild uveitis and nephritis that is self-limited; however, in this case, the symptoms were severe making it different from previous case reports. We present a case of a 29-year-old female with a history of cytomegalovirus (CMV) with a recent diagnosis of bilateral uveitis who was admitted for worsening systemic symptoms. Laboratory testing revealed acute renal insufficiency along with hematuria and proteinuria. A kidney biopsy revealed tubulointerstitial nephritis, and the patient was initiated on corticosteroids for the diagnosis of TINU. The patient's renal function recovered to baseline after a prolonged three-month course of systemic steroids but had a recurrence of her uveitis with steroid taper requiring initiation of steroid-sparing therapies. TINU syndrome should be considered in patients presenting with uveitis and renal dysfunction. Prompt diagnosis is necessary to preserve renal function with corticosteroids. The prognosis for patients with TINU is variable, with a frequently recurring and relapsing course. More research is needed to determine the optimal treatment.

Occult Uveitis-Glaucoma-Hyphema Syndrome Caused by PCIOL with Peripheral Capsular Tear.

Introduction: Uveitis-glaucoma-hyphema (UGH) syndrome is an infrequent but severe complication following intraocular lens implantation, characterized by anterior chamber inflammation and elevated intraocular pressure (IOP). This report presents a rare case of late-onset UGH syndrome induced by a well-positioned 1-piece posterior capsular intraocular lens (PCIOL) with a bulb of the haptics extruding through a peripheral capsular tear in a 90-year-old female, 17 years post-cataract surgery. Case Presentation: The patient presented with persistent blurred vision, recurrent anterior uveitis, and uncontrolled IOP despite medical therapy. Extensive evaluation, including ultrasound biomicroscopy, failed to identify the underlying cause, necessitating surgical intervention to control IOP. During concurrent goniotomy and canaloplasty, a PCIOL haptics was discovered protruding through a peripheral capsular tear, establishing the diagnosis. Following PCIOL-haptic amputation and goniotomy and canaloplasty, the patient experienced significant improvement in symptoms and IOP control, with complete resolution of UGH syndrome. Conclusion: This case highlights the necessity of considering atypical causes in persistent postoperative uveitis and IOP elevation and emphasizes the role of surgical intervention in managing complex cases.

AAV-mediated gene therapies for glaucoma and uveitis: are we there yet?

Glaucoma and uveitis are non-vascular ocular diseases which are among the leading causes of blindness and visual loss. These conditions have distinct characteristics and mechanisms but share a multifactorial and complex nature, making their management challenging and burdensome for patients and clinicians. Furthermore, the lack of symptoms in the early stages of glaucoma and the diverse aetiology of uveitis hinder timely and accurate diagnoses, which are a cause of poor visual outcomes under both conditions. Although current treatment is effective in most cases, it is often associated with low patient adherence and adverse events, which directly impact the overall therapeutic success. Therefore, long-lasting alternatives with improved safety and efficacy are needed. Gene therapy, particularly utilising adeno-associated virus (AAV) vectors, has emerged as a promising approach to address unmet needs in these diseases. Engineered capsids with enhanced tropism and lower immunogenicity have been proposed, along with constructs designed for targeted and controlled expression. Additionally, several pathways implicated in the pathogenesis of these conditions have been targeted with single or multigene expression cassettes, gene editing and silencing approaches. This review discusses strategies employed in AAV-based gene therapies for glaucoma and non-infectious uveitis and provides an overview of current progress and future directions.

CMV-Related Anterior Uveitis in a Mediterranean European Population: Clinical Features, Prognosis, and Long-Term Treatment Outcomes.

PURPOSE: To describe the spectrum of clinical features of cytomegalovirus-related anterior uveitis (CMV-AU) along with potential comorbidities, to calculate complication rates, and to determine risk factors and biomarkers affecting prognosis in a cohort of a Southern European Mediterranean population. MATERIALS AND METHODS: It is a retrospective, multicenter case series of consecutive patients with persisting hypertensive AU, unresponsive to topical steroids therapy, and CMV-positive essays from two uveitis referral centers were collected and analyzed. RESULTS: Fifty-seven eyes of 53 patients with polymerase chain reaction-verified CMV-AU over a period of 8 years were included with a mean age of 48 ± 18. Four presentation patterns were identified: 26.3% as Posner-Schlossman-like, 31.6% as chronic AU, 19.3% as presumed herpetic uveitis, 12.3% as Fuchs uveitis syndrome-like, and 10.5% without specific initial classification. About 15.8% received oral valganciclovir, 22.8% received topical valganciclovir, and 61.4% received both, for a mean duration of treatment of 44 months. AU recurrences were observed in 23 eyes with a mean of 1.5 (±1.5) recurrences per year. The only finding significantly associated with recurrence was the presence of posterior synechiae (PS) (p = 0.034). Fewer keratic precipitates (KPs) were indicative for the need of longer treatment, and endotheliitis was strongly associated with the need for filtration surgery. CONCLUSION: In this immunocompetent southern European population, four distinct clinical presentation patterns were further confirmed, and possible biomarkers such as PS, KPs, and endotheliitis were newly reported to influence treatment outcomes. Large-scale studies could provide a more effective customized treatment protocol.

Epidemiological, Clinical, and Therapeutic Profile of Uveitis in Multiple Sclerosis: A Multicenter Study.

INTRODUCTION: Uveitis may occur during approximately 1-3% of MS patients, corresponding to 10 times higher than in the general population. The development of uveitis is not currently considered as an inflammatory relapse of MS. There are no clinical guidelines for treating. MS with concomitant uveitis requiring systemic treatment. PURPOSES: To analyze clinical and therapeutic characteristics of uveitis in patients with MS and the impact of MS treatment on the progression of uveitis. MATERIALS & METHODS: We conducted a retrospective, observational, multicenter study in France about 54 patients. RESULTS: The form of MS most frequently associated with uveitis in our study was the relapsing-remitting form (85%). The mean time of onset of uveitis was 15 months before the diagnosis of MS. The most frequent form of uveitis was bilateral panuveitis (43%), non-granulomatous (61%), synechial (52%) and non-hypertonic (93%) with progressive onset (65%) and chronic course (66%). CONCLUSION: MS-associated uveitis occurs most frequently before the diagnosis of relapsing-remitting MS in the form of panuveitis or intermediate uveitis, which is mildly inflammatory and whose main complications are macular edema, cataract and venous vasculitis. Despite their chronicity, these uveitis cases have a good visual prognosis and teriflunomide appears to have a positive effect on progression.

Masquerade syndrome: A review of uveitic imposters.

Masquerade syndromes in uveitis are complex clinical conditions where non-inflammatory diseases mimic uveitic manifestations, often leading to diagnostic and therapeutic challenges. This review delves into the diverse spectrum of masquerade syndromes, categorizing them into neoplastic and non-neoplastic entities. We explore the prevalence of primary intraocular lymphoma, leukaemia, retinoblastoma, and other malignancies, as well as conditions like retinitis pigmentosa and endophthalmitis that can present as uveitis. Through detailed analysis of symptoms, diagnostic methods, and treatment approaches, the review emphasizes the importance of considering masquerade syndromes in differential diagnoses to prevent mismanagement. The synthesis of current knowledge aims to enhance clinicians' ability to discern these complex presentations, advocating for a multidisciplinary approach to diagnosis and care, thereby improving patient outcomes in cases of uveitic masquerade syndromes.

Vasoproliferative Tumor Secondary to Sarcoidosis-Associated Intermediate Uveitis.

We report the visual and clinical outcomes of a middle-aged woman who presented with exudative retinal detachment (ERD) secondary to a vasoproliferative tumor (VPT) in an eye with sarcoidosis-associated intermediate uveitis. A 55-year-old woman previously diagnosed with sarcoidosis presented with decreased vision in the left eye (LE). Visual acuity in the LE was counting fingers. She had active vitritis, and a peripheral retinal vascular mass was noted in the superotemporal periphery. The mass was associated with ERD involving the posterior pole. The patient was managed with systemic and intravitreal steroids, and cyclosporine was subsequently added as a steroid-sparing agent. Because of recurrence of ERD, the patient underwent pars plana vitrectomy, and cryotherapy and laser photocoagulation were applied to the VPT. Two months postoperatively, visual acuity in the LE improved to 6/10. There was marked regression of the VPT and total resolution of the ERD. In conclusion, we report a favorable visual and clinical outcome in a patient with VPT-associated ERD who responded to a combination of medical therapy and surgical intervention. VPT may lead to different remote complications, so timely diagnosis of these tumors and proper management of their complications is warranted.

Active toxoplasma chorioretinitis in immunocompromised patients: a case series.

Toxoplasma chorioretinitis (TC) can exhibit atypical features in immunocompromised patients including bilaterality, extensive spread, multifocal presentation, large areas of retinal necrosis without adjacent retinal scarring, and diffuse necrotizing retinitis resembling the viral retinitis that may cause confusion in the differential diagnosis. The aim of this study was to present the clinical features of four eyes of three immunocompromised patients with active toxoplasma chorioretinitis. Two of the patients were female and one, male. Two patients had hematological malignancies and the remaining patient was under adalimumab treatment for ankylosing spondylitis. Visual complaints began 10 days to four months prior to TC diagnosis. All four eyes had mild-to-moderate anterior chamber cells together with severe vitritis on slit-lamp examination while there were solitary chorioretinitis lesions on fundoscopy. Despite all patients were negative for anti-toxoplasma immunoglobulin M, all were positive for immunoglobulin G. All three patients were successfully treated with a combined treatment of systemic and intravitreal anti-toxoplasmic drugs. Clinicians should be cautious for the possible toxoplasma chorioretinitis besides the other infectious entities when a new uveitis episode is detected in an immunosuppressed patient in order to avoid misdiagnosis and thereby wrong treatment.

Ophthalmological and Neurological Findings in Patients with Idiopathic Uveitis Associated with Retinal Vasculitis and the Relation with the HLA DR15 Allele.

PURPOSE: In 15 patients with idiopathic uveitis associated with retinal vasculitis, HLA DRB1 gene testing was performed to detect a possible association. 11 patients tested positive and 4 negative for the HLA DRB1 × 15 allele. The presence of the HLA DRB1 × 15 haplotype might be associated with a higher susceptibility to develop Multiple Sclerosis (MS). METHODS: In this case series, we describe the ophthalmological and neurological findings in 10 HLA DR15-positive patients and 4 HLA DR15-negative patients that had neurological workup, including Magnetic Resonance Imaging (MRI) of the brain. RESULTS: All patients had granulomatous ocular inflammation with either panuveitis or intermediate uveitis. MRI of the brain showed white matter lesions in 13 patients (9/10 and 4/4 respectively) of which 4 patients were eventually diagnosed with MS (3/10 and 1/4 respectively). CONCLUSION: Although the majority of tested patients was carrying at least one HLA DRB1-15 allele, there was no difference in ophthalmological and neurological findings in both groups.

Sterile endophthalmitis after intravitreal injection of triamcinolone acetonide: case report and literature review.

Objectives: The first purpose is to present the diagnosis and therapeutic approach in a patient with sterile endophthalmitis associated with triamcinolone acetonide injection. The secondary objective is to assess the incidence of this complication and to summarize the risk factors described in the literature. Case presentation: A 76-year-old male patient presented for painless, unilateral, decreased visual acuity, four days after cataract surgery and simultaneously intravitreal triamcinolone acetonide injection for diabetic macular edema in the right eye. The diagnosis of sterile endophthalmitis was made. Eight days after the presentation, the symptoms subsided, the maximum corrected visual acuity reaching that before the procedures. Discussions: The incidence of sterile endophthalmitis varies in the literature between 0% and 23.8%. Visual prognosis is good, although the pathogenesis is not fully understood. Preservatives in injectable solutions have been suggested, however, there are studies in which inflammation was also present with preservative-free products. The particle size of triamcinolone was analyzed, demonstrating an association between smaller particles and an increased frequency of adverse reactions of this type. History of uveitis, posterior capsule rupture following cataract surgery, and Irvine-Gass syndrome are other associations described. Conclusion: The physiopathological mechanism of sterile endophthalmitis is not fully understood. However, the visual prognosis is good, the final vision being dependent on the underlying pathology.

Prevalence, clinical characteristics, and independent predictors of uveitic macular edema in an Asian population: a retrospective cohort study.

BACKGROUND: To determine the prevalence, clinical characteristics, and independent predictors of uveitic macular edema (UME) in patients with intermediate, posterior and panuveitis. METHODS: We retrospectively reviewed the records of patients with intermediate, posterior, and panuveitis who underwent macular assessment using optical coherence tomography between January 2015 and February 2020. The prevalence of UME and clinical characteristics of the patients were described. Predictors of UME were identified using multivariate regression analysis. RESULTS: A total of 349 patients were included. The mean age was 41 years, female: male ratio was 1.3:1. The prevalence of UME was 51.9%. UME was found in 33.9%, 56.9%, and 54.1% of the intermediate, posterior, and panuveitis cases, respectively. Among patients with UME, 47% had infectious uveitis, 32.6% had idiopathic uveitis, and 20.4% had immune-mediated uveitis. Diffuse macular edema was the most frequently observed pattern (36.5%). Multivariate analysis showed that factors independently associated with UME included age at uveitis onset (adjusted odds ratio [aOR] 1.01, 95% confidence interval [CI] 1.00-1.03, P = 0.036), PU and panuveitis compared with intermediate uveitis (aOR 2.09, 95% CI 1.14-3.86, P = 0.018), and infectious uveitis compared with noninfectious uveitis (aOR 2.13, 95% CI 1.34-3.37, P = 0.001). CONCLUSIONS: Increasing age at uveitis onset, posterior/panuveitis, and infectious etiology are predictive factors for UME in patients with intermediate, posterior and panuveitis.

The Frequency of Intraocular Pressure Elevation, Incidence of Secondary Glaucoma, and Surgical Treatment With Postoperative Complications in Pediatric and Adult Patients With Uveitis.

Purpose To determine the etiology and anatomic localization of uveitis, the frequency of intraocular pressure (IOP) elevation, and the type of secondary glaucoma and to assess the medical, surgical, and postoperative complications in adult and pediatric patients with acute or chronic uveitis. Methods A total of 307 eyes of 186 patients who were followed up in the Uvea-Behçet Unit of the Ophthalmology Department, Erciyes University, Turkey, were included in the study. Demographic, ocular, and systemic data were recorded; ophthalmological examinations were performed; and recurrences and complications of uveitis were identified. The eyes with IOP over 22 mmHg, types of secondary glaucoma, their etiologies, efficiency of medical and surgical treatments, and complications were recorded. Results The mean age was 33 ± 12 years (range: 6-65). Of the 186 patients, diagnoses were as follows: idiopathic uveitis in 84 (45.2%), Behçet disease in 65 (34.9%), ankylosing spondylitis in eight (4.3%), juvenile idiopathic arthritis in five (2.7%), herpetic keratouveitis in three (1.6%), Fuchs iridocyclitis in three (1.6%), Vogt-Koyanagi-Harada syndrome in three (1.6%), tuberculosis uveitis in three (1.6%), Crohn disease in three (1.6%), ocular toxoplasmosis in two (1.1%), multiple sclerosis in two (1.1%), Lyme disease in two (1.1%), rheumatoid arthritis in two (1.1%) and tubulointerstitial nephritis in one patient (0.5%). Secondary glaucoma was detected in 67 (21.9%) of 307 eyes, which developed in 13.7% and 26.8% of the eyes with acute and chronic uveitis, respectively. Of 67 eyes, it was open-angle glaucoma in 58 (86.5%), angle-closure glaucoma in six (9.0%), and neovascular glaucoma in three (4.5%). Control of IOP was achieved by medical therapy in 53 eyes (79.1%) and by surgery in 12 eyes (17.9%), whereas evisceration was required in two eyes (3.0%). Laser iridotomy was performed in four eyes (33.4%), trabeculectomy with mitomycin-C (MMC) in six eyes (50.0%), laser iridotomy plus trabeculectomy with MMC in one eye (8.3%), and express mini shunt implantation in one eye (8.3%). After surgery, IOP was controlled without anti-glaucomatous agents in six eyes (50%) and with anti-glaucomatous agents in the remaining six eyes (50.0%). Conclusion Secondary glaucoma is one of the most important complications of uveitis and may result in severe visual impairment. Early diagnosis and appropriate treatment can prevent these potential complications.

Rosai-Dorfman disease as chronic bilateral granulomatous anterior uveitis: A case report.

Rosai-Dorfman disease is a rare non-Langerhans cell histiocytosis disorder, also known as sinus histiocytosis, with massive lymphadenopathy. Rosai-Dorfman disease is classified into nodal and extranodal diseases. Ocular involvement is one of the common extranodal manifestations; however, uveitis manifestation is infrequent. We reported an interesting Rosai-Dorfman disease patient who presented with chronic bilateral granulomatous anterior uveitis with disc hyperemia and cystoid macular edema. She was a 60-year-old Thai female who had abnormal skin nodules on her back after multiple recurrent chronic anterior uveitis. The diagnosis has been made based on a constellation of the patient's information, physical examination, and histopathological results. We successfully treated the patient with topical steroids, posterior subtenon triamcinolone 20-mg injection, and methotrexate.

OCTN2- and ATB0,+-targeted nanoemulsions for improving ocular drug delivery.

Traditional eye drops are administered via topical instillation. However, frequent dosing is needed due to their relatively rapid precorneal removal and low ocular bioavailability. To address these issues, stearoyl L-carnitine-modified nanoemulsions (SC-NEs) were fabricated. The physicochemical properties of SC-NEs in terms of size, morphology, zeta potential, encapsulation efficiency, and in vitro drug release behavior were characterized. The cellular uptake and mechanisms of SC-NEs were comprehensively studied in human corneal epithelial cells and the stearoyl L-carnitine ratio in SC-NEs was optimized. The optimized SC-NEs could target the novel organic cation/carnitine transporter 2 (OCTN2) and amino acid transporter B (0 +) (ATB0,+) on the corneal epithelium, which led to superior corneal permeation, ocular surface retention ability, ocular bioavailability. Furthermore, SC-NEs showed excellent in vivo anti-inflammatory efficacy in a rabbit model of endotoxin-induced uveitis. The ocular safety test indicated that the SC-NEs were biocompatible. In general, the current study demonstrated that OCTN2 and ATB0,+-targeted nanoemulsions were promising ophthalmologic drug delivery systems that can improve ocular drug bioavailability and boost the therapeutic effects of drugs for eye diseases.

Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry.

VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03-5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up.