Management of Scleritis in Older Adults.

Scleritis, an inflammatory disease of the eye affecting scleral tissue, presents unique challenges in the older adult population. Unlike their younger counterparts, older individuals manifest a distinct spectrum of the disease with different underlying etiologies, co-morbidities, altered immune function, and an increased risk of systemic side effects from medication choices. Addressing these complexities necessitates a comprehensive and multidisciplinary approach. Treatment of choice will depend on any underlying cause but generally involves non-steroidal anti-inflammatory drugs, systemic or local corticosteroids, and potentially disease-modifying anti-rheumatic drugs. Utilization of these therapeutic agents in older adults warrants careful consideration because of their potential side-effect profiles. This article critically examines the specific concerns for the use of these drugs in older patients and reviews the existing literature on their use in this specific cohort.

Surgically Induced Scleritis Associated With Suture Hypersensitivity Following Strabismus Surgery.

A 67-year-old woman with history of mild suture hyper-sensitivity presented with localized scleritis after strabismus surgery. After infection was ruled out, the patient was prescribed topical and systemic non-steroidal anti-inflammatory drugs and systemic steroids, which led to full clinical resolution. [J Pediatr Ophthalmol Strabismus. 2024;61(1):e4-e6.].

ANCA-associated scleritis: impact of ANCA on presentation, response to therapy and outcome.

OBJECTIVES: To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests. METHODS: This retrospective multicentre case-control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests. RESULTS: A total of 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022 were included. The median follow-up was 28 months (IQR 10-60). The median age at diagnosis was 48 years (IQR 33-60) and 75% were females. Scleromalacia was more frequent in ANCA-positive patients (P = 0.027) and 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% vs 34%; P < 0.001), and rituximab (P = 0.03) and had a lower remission rate after the first- and second-line treatment. Systemic ANCA-associated vasculitis (AAV) occurred in 30.7% of patients with PR3- or MPO-ANCA, after a median interval of 30 months (IQR 16.3-44). Increased CRP >5 mg/l at diagnosis was the only significant risk factor of progression to systemic AAV [adjusted hazard ratio 5.85 (95% CI 1.10, 31.01), P = 0.038]. CONCLUSION: Isolated ANCA-associated scleritis is mostly anterior scleritis with a higher risk of scleromalacia than ANCA-negative idiopathic scleritis and is more often difficult to treat. One-third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV.

Posterior scleritis following COVID-19 vaccination or infection simulating uveal melanoma in 8 consecutive patients.

OBJECTIVES: To determine clinical features and outcomes of posterior scleritis masquerading as uveal melanoma following vaccination against COVID-19 and/or COVID-19 infection. SUBJECTS/METHODS: All patients with posterior scleritis referred to our service to rule out intraocular tumour between February 2021 and June 2022, who previously had COVID-19 vaccination and/or infection (n = 8). A retrospective detailed review of patient charts and imaging was carried out. RESULTS: Previous COVID-19 vaccination was documented in 6 patients (75%) and previous COVID-19 infection and vaccination in 2 patients (25%). Demographic features included mean age of 59 years (median 68, range 5-86 years), white race (n = 7, 87%), and male sex (n = 5, 63%). Mean visual acuity at presentation was 0.24 LogMAR (median 0.18, range 0.0-0.70). The main presenting symptom was blurred vision with pain (n = 5, 63%). Features that suggested scleritis and not uveal melanoma included pain (n = 6, 75%), anterior scleritis (n = 3, 38%), disc oedema (n = 1, 13%), choroidal detachment (n = 3, 38%), choroidal folds (n = 3, 38%), diffusely thickened scleral wall on ultrasonography (n = 2, 25%), Tenon's oedema (n = 5, 63%), and scleral nodule with medium/high internal reflectivity on ultrasonography (n = 4, 50%). Follow-up information at mean of 2 months (range 0.25-7 months) revealed visual acuity at date last seen was mean 0.30 LogMAR (median 0.29, range 0.0-0.54). By 2 months, resolution of "tumour" was noted in 5/6 (83%) patients with follow-up. CONCLUSIONS: Posterior scleritis following COVID-19 vaccination and/or infection can masquerade as choroidal melanoma. At 2 months duration, partial or complete resolution of features with minimal visual consequence was noted.

Scleral changes in systemic lupus erythematosus patients using swept source optical coherence tomography.

Purpose: This study aims to examine scleral thickness in patients with systemic lupus erythematosus (SLE) without clinically evident scleritis and episcleritis, utilizing swept-source optical coherence tomography (SS-OCT). Methods: This cross-sectional single center study compared scleral thickness (Nasal scleral thickness 1mm, 2mm, 3mm, 6mm from scleral spur; Temporal scleral thickness 1mm, 2mm, 3mm, 6mm from scleral spur) in 73 SLE patients without clinically evident scleritis and episcleritis and 48 healthy volunteers with SS-OCT. Further, we investigated the correlation between scleral thickness in SLE patients and various parameters including laboratory markers, disease duration, disease activity, and organ involvement. Results: Across all measured sites (nasal scleral thickness at distances of 1mm, 2mm, 3mm, and 6mm from the scleral spur, and temporal scleral thickness at the same distances), the scleral thickness in the SLE group was significantly greater than that in the control group (all p-values <0.001). SLE patients with a disease duration of 5 years or less exhibited a higher scleral thickness compared to those with a more prolonged disease duration. Patients with a higher erythrocyte sedimentation rate (ESR) had a thinner temporal scleral thickness. However, no significant associations were identified between scleral thickness and disease activity, organ involvement, or other laboratory markers. Conclusion: Scleral thickness measured by SS-OCT was higher in SLE patients than healthy controls. Changes in scleral thickness in SLE patients are related to disease duration and ESR. SS-OCT can detect asymptomatic structural changes in SLE patients and may be a useful tool in the evaluation of early scleral abnormality.

Nodular Posterior Scleritis With Associated Choroiditis Masquerading As A Choroidal Tumour: A Case Report.

A case of nodular posterior scleritis in a 25-year-old male who presented with a 14-day history of unilateral decline in vision, pain, and redness in his right eye. Slit lamp examination of the right eye revealed dilated episcleral vessels present nasally as well as a choroidal mass at the nasal periphery of the fundus, associated with choroidal oedema. Systemic evaluation and imaging of the choroidal mass were performed to rule out choroidal tuberculoma and choroidal metastasis. Ultrasound B-scan of the right eye showed marked thickening of the nasalsclera resulting in sympathetic choroidal oedema without the characteristic T-sign. Nodular posterior scleritis with associated choroiditis, was diagnosed without any underlying systemic illness. The patient was immediately started on systemic steroids and later on subcutaneous Methotrexate as advised by the rheumatologist, to which he responded well and his vision significantly improved from 6/60 to 6/9, gradually during his treatment course.

Epiescleritis y edema periorbitario secundario a síndrome VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) / Episcleritis and periorbital edema secondary to VEXAS syndrome

El síndrome vacuolas, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) es una nueva entidad autoinflamatoria descrita recientemente, producida por una mutación del gen UBA-1. Entre los síntomas más frecuentes están la fiebre, las citopenias, la policondritis, los infiltrados pulmonares y hasta en un 40% afectación ocular en forma de edema periorbitario, uveítis, epiescleritis, escleritis y vasculitis retiniana. Los pacientes responden a altas dosis de corticoterapia, sin embargo muchos terminan siendo refractarios a las mismas y a los inmunosupresores clásicos. Se describe el caso de un paciente varón de 77 años con afectación ocular en forma de epiescleritis y edema periorbitario que posteriormente fue diagnosticado de síndrome VEXAS. El paciente, tras fracasar al tratamiento con inmunosupresores, en la actualidad está en tratamiento con esteroides orales y tocilizumab. Los especialistas en oftalmología deben estar al corriente de la afectación oftalmológica de las enfermedades autoinflamatorias, y en especial de esta nueva entidad descrita, como es el síndrome VEXAS (AU)

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly described autoinflammatory entity caused by a UBA-1 gene mutation. Among the most frequent symptoms it produces fever, cytopenias, polychondritis, pulmonary infiltrates and up to 40% ocular involvement such as periorbital edema, uveitis, episcleritis, scleritis and retinal vasculitis. Patients respond to high doses of corticosteroids, however, many end up being refractory to them and to the classic immunosuppressants. We described the case of a 77-year-old male patient with ocular involvement in the form of episcleritis and periorbital edema who was later diagnosed with VEXAS syndrome. The patient, after failing treatment with immunosuppressants, is currently receiving treatment with oral steroids and tocilizumab. Ophthalmologist must be aware of the ophthalmological affectation of autoinflammatory diseases and especially of this new entity described as the VEXAS syndrome (AU)

Episcleritis and periorbital edema secondary to VEXAS syndrome.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly described autoinflammatory entity caused by a UBA-1 gene mutation. Among the most frequent symptoms it produces fever, cytopenias, polychondritis, pulmonary infiltrates and up to 40% ocular involvement such as periorbital edema, uveitis, episcleritis, scleritis and retinal vasculitis. Patients respond to high doses of corticosteroids, however, many end up being refractory to them and to the classic immunosuppressants. We described the case of a 77-year-old male patient with ocular involvement in the form of episcleritis and periorbital edema who was later diagnosed with VEXAS Syndrome. The patient, after failing treatment with immunosuppressants, is currently receiving treatment with oral steroids and tocilizumab. Ophthalmologist must be aware of the ophthalmological affectation of autoinflammatory diseases and especially of this new entity described as the VEXAS Syndrome.

Acute-onset surgically induced necrotizing scleritis after strabismus surgery.

Surgically induced necrotizing scleritis (SINS) is an uncommon but devastating complication that may occur days to years after ocular surgery. We report the case of a 32-year-old man who underwent uncomplicated strabismus surgery for large-angle exotropia and developed SINS characterized by painless scleral inflammation, choroidal exposure, and globe ectasia within days of surgery. Work-up revealed no associated infectious process or underlying systemic inflammatory condition. Clinical resolution occurred with oral immunosuppression alone, without need for graft.

Comprehensive profiling of extracellular vesicles in uveitis and scleritis enables biomarker discovery and mechanism exploration.

BACKGROUND: Uveitis and posterior scleritis are sight-threatening diseases with undefined pathogenesis and accurate diagnosis remains challenging. METHODS: Two plasma-derived extracellular vesicle (EV) subpopulations, small and large EVs, obtained from patients with ankylosing spondylitis-related uveitis, Behcet's disease uveitis, Vogt-Koyanagi-Harada syndrome, and posterior scleritis were subjected to proteomics analysis alongside plasma using SWATH-MS. A comprehensive bioinformatics analysis was performed on the proteomic profiles of sEVs, lEVs, and plasma. Candidate biomarkers were validated in a new cohort using ELISA. Pearson correlation analysis was performed to analyze the relationship between clinical parameters and proteomic data. Connectivity map database was used to predict therapeutic agents. RESULTS: In total, 3,668 proteins were identified and over 3000 proteins were quantified from 278 samples. When comparing diseased group to healthy control, the proteomic profiles of the two EV subgroups were more correlated with disease than plasma. Comprehensive bioinformatics analysis highlighted potential pathogenic mechanisms for these diseases. Potential biomarker panels for four diseases were identified and validated. We found a negative correlation between plasma endothelin-converting enzyme 1 level and mean retinal thickness. Potential therapeutic drugs were proposed, and their targets were identified. CONCLUSIONS: This study provides a proteomic landscape of plasma and EVs involved in ankylosing spondylitis-related uveitis, Behcet's disease uveitis, Vogt-Koyanagi-Harada syndrome, and posterior scleritis, offers insights into disease pathogenesis, identifies valuable biomarker candidates, and proposes promising therapeutic agents.

Systemic Vasculitis and Its Association with the Eye.

Vasculitis is a group of diseases characterized by the inflammation of the blood vessel walls. They are classified according to the size of the main vessel involved: large vessel, medium vessel, and small vessel vasculitis. Ophthalmic manifestations are quite common in most of these diseases. Episcleritis and scleritis are the most prevalent manifestation of vasculitis. However, there are certain ocular features characteristic of specific vasculitis entities. Given the severity and potential life-threat of these diseases, knowledge of the ocular manifestations is mandatory for the ophthalmologists.

[Scleritis and episcleritis]. / Sclérite et épisclérite.

Scleritis and episcleritis are rare ocular inflammatory diseases but deserve to be known by internists because of their frequent association with systemic autoimmune diseases. It is important to distinguish them between because their prognosis, therapeutic management and potential complications are very different. Episcleritis represents a superficial ocular inflammation with usually benign visual prognosis, no complication with local treatment, and is associated with a systemic autoimmune disease in rare cases. In contrast, scleritis is a potentially serious ophthalmological condition that can threaten the visual prognosis in the absence of appropriate systemic treatment. It is associated with an underlying disease in 40-50% of cases, in particular a systemic autoimmune disease (25-35% of cases) or an infectious cause (5-10% of cases). Rheumatoid arthritis and systemic vasculitides, particularly antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, are the main autoimmune causes of scleritis and episcleritis. Scleritis can reveal the underlying autoimmune disease and requires systematic etiological investigations. Aggressive, complicated, refractory forms or those associated with a systemic autoimmune disease require glucocorticoids or even immunosuppressants, and close collaboration between ophthalmologists and internists is required. The development of biologic agents offers new effective therapeutic tools in the management of these difficult cases.

Panscleritis After Blunt Ocular Trauma in A Child with Epididymitis.

We reported an 8-year-old boy with panscleritis in left eye and right epididymitis after falling on the ground. Etiologic diagnosis played a key role in this case. Systemic examinations ruled out systemic autoimmune diseases, tumors, and infections as the cause of scleritis and suggested that the disease was caused by a local delayed-type hypersensitivity (DTH) induced by ocular trauma and was non-infectious. Still, the right epididymitis was infectious. Both conditions were treated successfully using steroids and antibiotics, respectively. Thus, early etiologic diagnosis and reasonable treatment are crucial to prevent visual loss.

Inflammatory eye disease for rheumatologists.

PURPOSE OF REVIEW: This review provides a framework for understanding inflammatory eye disease diagnosis, differential diagnosis, and management for rheumatologists. Uveitis, scleritis, episcleritis, peripheral ulcerative keratitis, and orbital inflammation are all discussed. The goal is to facilitate the development of approaches to inflammatory eye diseases that will help rheumatologists co-manage these patients with eye care providers specializing in ocular inflammation. RECENT FINDINGS: In recent years, studies have aimed to advance biologic treatments and define standard-of-care therapy. Inflammatory eye diseases are highly heterogeneous and often rare, which poses significant challenges to their research and the interpretation of existing data. To date, glucocorticoids, mycophenolate, methotrexate, and TNF inhibitors remain the mainstay of treatment options for many of these diseases. SUMMARY: Patients with inflammatory eye diseases require multidisciplinary care for best outcomes, frequently including rheumatologists. Understanding the differentials, diagnostics, and treatment are essential to preserving vision in these patients. The diverse nature of the disease processes within this field requires focusing on specific disease phenotypes and endotypes in research and clinical practice.

Scleritis and Episcleritis following Coronavirus Disease (COVID-19) Vaccination.

INTRODUCTION: Coronavirus disease (COVID-19) vaccines have been reported to have ocular side effects including scleritis and episcleritis. PURPOSE: To report scleritis and episcleritis within a month following administration of COVID-19 vaccine. METHODS: Retrospective case series. RESULTS: 15 eyes of 12 consecutive patients with scleritis and episcleritis from March 2021 to September 2021 were included. The mean time of onset of symptoms in patients with scleritis was 15.7 days (range, 4-30) and for episcleritis it was 13.2 days (range 2-30). Patients received COVISHIELD™ (10 patients) and COVAXIN™ (2 patients). Five patients had denovo inflammation and seven had recurrent inflammation. Episcleritis patients were treated with topical steroids and systemic COX2 inhibitors while patients with scleritis were treated with topical steroids/oral steroids/antiviral medications depending on the aetiology. CONCLUSION: Scleritis and episcleritis following COVID-19 vaccination are milder and do not require intensive immunosuppression except in rare cases.

The ocular manifestations of relapsing polychondritis.

PURPOSE: Relapsing polychondritis (RPC) is a rare, multi-system, inflammatory disorder. Ocular disease is estimated to occur in 14-67% of patients with RPC, and any ocular structure can be affected. Published case reports and series of RPC were analysed to determine the frequency and nature of the ocular manifestations of RPC, including the age and gender distribution. METHODS: A literature search of the MEDLINE database for case reports and series on RPC was conducted in October 2021 using search terms [relapsing polychondritis (MeSH Major Topic)] OR [relapsing polychondritis (Title/Abstract)]. Articles were included if the diagnosis of RPC was confirmed using established diagnostic criteria and if the paper described the clinical features of patients with RPC. RESULTS: 546 articles (454 case reports and 92 case series) described the clinical features in a total of 2414 patients with RPC. 49% of patients with RPC had ocular involvement, and this was a presenting feature in 21%. The most common ocular manifestations were scleritis (32%), episcleritis (31%) and uveitis (23%). CONCLUSION: Many patients with RPC will be seen by an ophthalmologist during the course of their disease. Knowledge and awareness of RPC and its ocular manifestations is therefore essential to enable the ophthalmologist to make the diagnosis.

Necrotizing Scleritis and Rheumatoid Arthritis: A Clinical Case Report Supported by A Brief Review with Risk of Bias Analysis.

INTRODUCTION: Necrotizing scleritis (NS) presents 30%-40% as having a systemic autoimmune condition. OBJECTIVE: To present a clinical case report and a systematic review of necrotizing scleritis with ocular manifestation as the first sign of rheumatologic disease. METHODS: The present study was elaborated according to the rules of CARE. CASE REPORT: A female patient, 63 years old, a white, administrative assistant, presented irritation, low visual acuity (LVA) in the left eye (LE), and headache. Biomicroscopy (BIO) in the right eye (RE) was normal, and the LE showed hyperemia and scleral thinning. After 1 month, the patient returns without signs of infectious diseases in the exams, and after a rheumatological evaluation with a diagnosis of rheumatoid arthritis, methotrexate and prednisone are prescribed. After 2 months, she relapsed and started treatment with anti-TNF, with remission after the 4th dose. After 1 year, she evolved with LVA in LE. RESULTS: A total of 244 articles were found, 104 articles were evaluated and 10 were included in the brief review. The symmetrical Funnel Plot does not suggest a risk of bias. CONCLUSION: Both in the present case report and the literary findings, it was evidenced that the ophthalmologic findings may precede the systemic changes of the disease for the early diagnosis of rheumatoid arthritis.

Clinical profile and management of granulomatosis with polyangiitis-associated scleritis from a tertiary care hospital in South India.

Purpose: To describe the clinical features and management of patients with scleritis associated with granulomatosis with polyangiitis (GPA) at a tertiary eye care center in South India. Methods: The clinical profile and management of patients presenting to a tertiary eye care center in South India with scleritis secondary to GPA from 2003 to 2021 were analyzed retrospectively. Scleritis was classified into anterior diffuse, nodular, and necrotizing scleritis with inflammation according to Watson and Hayreh's classification. Demographic characteristics, clinical features, anti-neutrophil cytoplasmic antibody (ANCA) positivity, treatment response, ocular complications, and status at the last follow-up were analyzed. Statistical analysis of data was performed using Microsoft Excel 2019. Results: Nineteen eyes of 17 patients (15 cytoplasmic staining ANCA [c-ANCA], two p-ANCA positive) were included. Fifteen eyes had necrotizing scleritis, two had diffuse anterior scleritis, and two had nodular scleritis. Remission was induced using a combination of steroids and cyclophosphamide or rituximab. Maintenance therapy was instituted using tapering steroids and immunosuppressants like cyclophosphamide, mycophenolate mofetil, methotrexate, or rituximab. Three eyes required a scleral patch graft. Fourteen patients had good anatomical and visual outcomes, and three were lost to follow-up. Conclusion: GPA is a rare disease, while it is the most common ANCA-associated vasculitis with scleritis. As scleritis may be the presenting sign of the disease, ophthalmologists must be aware of the various features suggestive of GPA. GPA-associated scleritis can have a good prognosis when diagnosed promptly and managed aggressively in the acute stage, and remission is maintained with adequate systemic immunosuppression.