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Necrotizing scleritis is the most destructive and vision-threatening form of scleritis. Necrotizing scleritis can occur in systemic autoimmune disorders and systemic vasculitis, as well as following microbial infection. Rheumatoid arthritis and granulomatosis with polyangiitis remain the commonest identifiable systemic diseases associated with necrotising scleritis. Pseudomonas species is the most common organism causing infectious necrotizing scleritis, with surgery the most common risk factor. Necrotizing scleritis has the highest rates of complications and is more prone to secondary glaucoma and cataract than other phenotypes of scleritis. The differentiation between non-infectious and infectious necrotizing scleritis is not always easy but is critical in the management of necrotizing scleritis. Non-infectious necrotizing scleritis requires aggressive treatment with combination immunosuppressive therapy. Infectious scleritis is often recalcitrant and difficult to control, requiring long-term antimicrobial therapy and surgical debridement with drainage and patch grafting due to deep-seated infection and the avascularity of the sclera.

Immunoglobulin A nephropathy (IgAN) is a rare but important systemic disease with or without ocular manifestations. We describe 4 cases of IgAN presenting with scleritis and review the various ocular manifestations in patients with IgAN. We found 55 cases with ocular manifestations in patients with prior or newly-diagnosed IgAN described in 38 publications. The most common ocular manifestations of IgAN were episcleritis (23.6%), scleritis (16.4%), hypertensive retinopathy or retinal vasculopathy (20.0%), and uveitis (14.5%). The median age at presentation was 36.5 years, with 54.5% female patients. 61.8% had history of IgAN prior to ocular involvement, while 29.1% had ocular presentations as the first manifestation of IgAN. The majority received systemic corticosteroids and/or immunosuppressants. Additionally, we report 4 women with anterior scleritis and previous diagnosis of IgAN. All 4 were treated with topical and systemic corticosteroids. Three out of 4 patients had no recurrence for at least 1 year since the first presentation. IgAN is a rare but important systemic association to be considered in ocular inflammatory conditions. Timely recognition and comanagement of the disease with nephrologist could reduce disease morbidity., Competing Interests: Disclosures The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (Copyright © 2022. Published by Elsevier Inc.)

Purpose: The purpose of this study was to report a case of infective necrotizing scleritis following XEN Gel Stent with mitomycin-C., Methods: Case report. This is a case report of a 68-year-old woman., Results: XEN Gel Stent glaucoma surgery enhanced with mitomycin-C 0.04% and combined with cataract surgery was performed at a regional center to manage the patient's primary open-angle glaucoma. Past medical history was significant for rheumatoid arthritis requiring treatment with methotrexate and adalimumab. Periocular pain and swelling developed 14 months after the initial operation, followed by a rapid deterioration of visual acuity to 20/60, intraocular pressure of 4 mm Hg, and worsening pain 5 months later. On initial presentation to Sydney Eye Hospital, 180 degrees of scleral necrosis was evident with a moderate anterior segment inflammatory reaction and a large temporal choroidal effusion due to hypotony. Empirical hourly topical ofloxacin and cephalothin 5% drops, with oral moxifloxacin, were initiated. Conjunctival swab grew Staphylococcus aureus and Staphylococcus lugdunensis. Significant clinical improvement occurred, but the XEN Gel Stent became exposed after 9 days of treatment with worsening hypotony. Urgent surgical revision was performed to remove the XEN Gel Stent and apply a tutoplast plug with overlying amniotic membrane graft. Intraocular pressure gradually improved over 6 weeks to 15 mm Hg with reversal of hypotonous changes, and visual acuity stabilized at 20/40., Conclusions: To our knowledge, this is the first reported case of necrotizing scleritis following XEN Gel Stent insertion. It is a reminder that infection should always be the primary differential diagnosis in patients with surgical-induced necrotizing scleritis., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Abstract: Scleritis is a rare, vision-threatening inflammation of the sclera that is often associated with life-threatening systemic illnesses. Rheumatoid arthritis remains the most common associated systemic rheumatic disease and the commonest systemic association of scleritis. Granulomatosis with polyangiitis is the most common cause of vasculitis-associated scleritis. The etiopathogenesis of scleritis remains unclear, but can be immune complex-mediated or due to a local delayed hypersensitivity reaction. Scleritis can involve either the anterior or posterior sclera, and has a wide spectrum of clinical presentations. Among the subtypes of scleritis, necrotizing scleritis has an increased risk of complications and is more commonly associated with anterior uveitis and peripheral ulcerative keratitis. Posterior scleritis is often not diagnosed or missed due to its subtle clinical signs and protean manifestations. Meticulous history taking, detailed ocular examination, and a targeted array of investigations with a multi-disciplinary approach to find any underlying systemic disease are crucial for the management of a case of scleritis. Corticosteroids remain the mainstay of short-term treatment of scleritis; mild to moderate scleral inflammation may respond well to treatment with nonsteroidal antiinflammatory drug or topical corticosteroid. Corticosteroid-sparing immunosuppressive therapies are useful in cases with an inadequate response or failure to provide long-term control of inflammation, and to prevent recurrence of scleritis. Biologic agents are increasingly used in the management of scleritis, not responding to the conventional therapies. This review provides an overview of the various subtypes of scleritis and its systemic associations and evaluates current trends in the diagnosis and management of noninfective scleritis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 Asia-Pacific Academy of Ophthalmology. Published by Wolters Kluwer Health, Inc. on behalf of the Asia-Pacific Academy of Ophthalmology.)

Purpose : To examine the spectrum of scleritis in four tertiary institutions across the Asia-Pacific. Methods : Clinical records from 354 patients were reviewed from centers in Australia, Singapore, and India, excluding those with insufficient data ( n  = 24). Results : Indian patients presented younger (41.5 ± 13.4 years) than Australians (50.8 ± 17.5) and Singaporeans (48.6 ± 15.9), with fewer women (49% vs 62%/57%). Diffuse disease was universally most common. Autoimmune and infectious disease proportions were similar in Australia (31%/10.3%) and Singapore (27.5%/8.3%) but reversed in India (8.3%/30%). Necrotizing scleritis was most frequently associated with infection (27.3%). Presumed ocular tuberculosis accounted for 75% of infectious cases in India. Posterior scleritis had the highest complication rate (82.4%) and immunosuppressants used per patient (0.98 ± 0.31 95% CI). Conclusions : Clinical presentations of scleritis vary across the Asia-Pacific, particularly in endemic regions for tuberculosis such as India, where it affects younger men with a predominance of nodular and infectious disease.

Objective: To ascertain whether patients on long-term systemic immunosuppressive therapy for inflammatory eye disease (IED) are at increased risk of malignancy., Design: A single-center, retrospective cohort study., Participants: We included 190 adults with IED treated with corticosteroids only (n = 58) or systemic immunosuppression (n = 132) for ≥6 months between 1985 and 2007. Immunosuppressed patients were treated with antimetabolites, T-cell inhibitors, and/or alkylating agents., Methods: Incident malignancies were ascertained by self-report and confirmed by medical record review. Multiple malignancies in a single patient were counted, except for nonmelanoma skin cancer (NMSC), where only the first was counted. Standardized incidence ratios (SIRs) were calculated by malignancy type. Cox regression models were used to compare malignancy incidence by treatment type., Main Outcome Measures: Risk of malignancy relative to the general population and within the cohort., Results: During a median 7.34 years of follow-up, 25 malignancies were observed in 17 patients, namely, 2.10 per 100 person-years and 0.43 per 100 person-years in the immunosuppressed and corticosteroid only groups, respectively. In the immunosuppressed group, the most common malignancies were NMSC (n = 11) and non-Hodgkin's lymphoma (NHL; n = 4) and malignancy risk was significantly increased compared with the general population for any malignancy (SIR, 4.39; 95% CI, 2.78-6.59) and for any malignancy excluding NMSC (SIR, 4.16; 95% CI, 1.67-8.57). Significantly elevated SIRs were observed for NMSC and NHL in those treated with immunosuppressive agents. Compared with the corticosteroid treatment-only group, the immunosuppressed group was at an increased risk of any malignancy (adjusted hazard ratio, 4.36; 95% CI, 1.02-18.7), but not first malignancy (n = 17; adjusted hazard ratio, 2.56; 95% CI, 0.57-11.5). No cancer-related deaths were observed., Conclusions: Our findings suggest that patients with IED treated with systemic immunosuppressive therapy are at increased risk of malignancy; however, the increase in absolute risk was modest. The types of malignancies observed at excess risk are similar to those observed in solid organ transplant recipients and patients with autoimmune diseases treated with systemic immunosuppression. Immunosuppressive therapy remains an important treatment modality in IED; however, patients may benefit from targeted malignancy-prevention strategies and long-term clinical follow-up. These findings require validation by a prospective, long-term, population-based cohort study., (Copyright © 2015 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Scleritis is an uncommon disease characterized by inflammation of the sclera and adjacent ocular structures. Recent studies have led to significant progress in understanding the epidemiology, immunopathogenesis, severity assessment, treatment, and prognosis of this potentially sight threatening disease. Despite these advances, significant challenges remain regarding our understanding of the mechanisms of scleral destruction and inflammation, and the rational approach to treatment. Information from studies in associated systemic diseases and vasculitis and a small number of studies of ocular tissue has revealed the prominent role of T and B cells, autoantibodies, immune complexes, and cytokines, such as TNF-alpha. These studies have prompted clinical trials that have demonstrated the effectiveness of anti-TNF, anti-B cell therapy, systemic immunosuppression, and more recently the use of local sub-conjunctival steroid treatment.

Scleritis is a heterogeneous group of diseases characterized by inflammation of the sclera, which may be due to local or systemic infections or immune mediated diseases. Numerous studies over the last decade have lead to significant progress in understanding the pathogenesis and treatment of this severe and potentially blinding disease. Immunological investigations of non-infectious scleritis and associated diseases have indicated that scleritis is an autoimmune disease and studies on the nature of the local inflammatory response have revealed the prominent role of T and B cells and cytokines, such as TNF-alpha, which in turn has resulted in clinical trials showing the effectiveness of local steroid treatment, anti-TNF and anti-B cell therapy. The widespread use of imaging has led to the realization that posterior scleritis is more common than previously recognized and testing for ANCA antibodies has revealed the prominent role of immune mechanisms in a subset of patients with scleritis and associated systemic vasculitis., (Crown Copyright © 2013. Published by Elsevier Ltd. All rights reserved.)

Purpose: We sought to characterize the long-term outcomes and complications of subconjunctival triamcinolone acetonide injection (STI) for non-necrotizing, noninfectious anterior scleritis., Design: Retrospective, interventional, noncomparative, multicenter study., Participants: Sixty-eight eyes of 53 patients from 9 participating hospitals in the United States, Singapore, and Australia. Only eyes with 6 or more months of follow-up were included., Intervention: Subconjunctival injection of 2 to 8 mg of triamcinolone acetonide was administered to eyes with non-necrotizing, noninfectious anterior scleritis., Main Outcome Measures: Resolution of signs and symptoms, time to recurrence of scleritis, and side effect profile., Results: Median follow-up was 2.3 years (range, 6 months to 8.3 years). Sixty-six eyes (97.0%) experienced improvement of signs and symptoms after 1 injection. Twenty-four months after a single injection, 67.6% of eyes remained recurrence-free, whereas at 48 months, 50.2% were recurrence-free. Some 55.0% of patients who had adverse effects from systemic medications were off all systemic medications at last follow-up; 55.0% of patients who were taking systemic medications at the time of first triamcinolone acetonide injection were not taking prednisone and immunosuppressants at this time; 76.2% of patients still requiring systemic agents had associated systemic disease. Fourteen eyes (20.6%) had ocular hypertension not requiring intraocular pressure (IOP)-lowering therapy. Two eyes (2.9%) were treated with topical IOP-lowering agents alone, and 2 eyes required surgical intervention for glaucoma. None developed scleral necrosis or melt., Conclusions: This retrospective, international study carried out at 9 hospitals suggests that STI can treat non-necrotizing, noninfectious anterior scleritis with side effects limited to elevated IOP in a few patients. Although no cases of scleral melt or necrosis were observed, we cannot definitively conclude that this may not occur after STI. Intraocular pressure should be closely monitored after STI. Subconjunctival triamcinolone acetonide injection may be useful as adjuvant therapy or to decrease systemic medication burden., Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references., (Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Scedosporium prolificans is an unusual infection, especially following pterygium surgery. A 63-year-old man who developed an uncomfortable, red right eye 10 years after pterygium surgery with adjunctive mitomycin C is described. To the best of the authors' knowledge, this is the first reported case of S. prolificans sclerokeratitis following pterygium surgery with adjunctive mitomycin C.

Scleritis is typically a severe painful inflammatory process centered in the sclera that may involve the cornea, adjacent episclera, and underlying uvea; it poses a significant threat to vision. Careful clinical history taking, detailed ocular examination, appropriate investigation for ocular disease with or without underlying systemic disease, and timely intervention with the use of immunosuppressant drugs when necessary, has improved the long-term outcome for patients with this disease.

Necrobiotic xanthogranuloma (NXG) is a rare systemic disease, that commonly manifests with orbital and ocular adnexal involvement, presenting with periocular yellow papules and plaques that may ulcerate. Periorbital skin lesions are a hallmark of the disease, and in their absence the diagnosis may be delayed, preventing prompt systemic evaluation and appropriate treatment of this condition. We report a unique case of a 58-year-old female patient with NXG that presented with severe bilateral necrotizing scleritis, left orbital mass and no cutaneous manifestations of NXG. This case highlights the importance of considering NXG, despite absence of skin lesions, when other ophthalmological manifestations are present. Recognition of NXG is crucial due to the high lifelong risk of developing hematological malignancy. [ABSTRACT FROM AUTHOR]

Scleritis is a severe chronic inflammation of the eye wall. High-dose corticosteroids and other immunosuppressive drugs are often required to control the inflammatory process. With the development of new and potentially more effective treatment modalities for scleritis has emerged the need for an accurate and reproducible system for quantifying the severity of scleritis and evaluating the response of individual patients to treatment. We have developed a quantitative scoring system, based on common clinical signs of scleritis, and evaluated it in 24 patients with scleritis. Our results indicate that this system is simple, rapid, reproducible and useful in grading the severity of scleritis and in predicting the response of patients to systemic immunosuppressive therapy. [ABSTRACT FROM AUTHOR]

Scleritis is an important, severely destructive, chronic inflammatory disorder affecting the eye wall. It presents a difficult management problem, often requiring high-dose systemic corticosteroid therapy or other immunosuppressive regimens to control the inflammatory response. A quantitative scleritis scoring system has been developed and its application to the assessment and management of scleral disease is discussed. This paper reviews current concepts in the management of scleral disease with emphasis on newer treatment modalities, such as pulse therapy with intravenously administered methylprednisolone or cyclophosphamide, and the use of orally administered cyclosporin. [ABSTRACT FROM AUTHOR]