Idiopathic Orbital Inflammation Associated With Necrotizing Scleritis and Temporal Bone Inflammation

Kasi, Sundeep; Kim, H. Jane; Basham, Ryan; Cunningham, Emmett T.; Sy, Aileen; Lustig, Larry; Kersten, Robert C.

doi:10.1097/iop.0000000000000251

Cited by 7 publications

(11 citation statements)

References 8 publications

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“…Prior nomenclature has is likely due to induction of apoptosis secondary to antibody dependent, cell-mediated toxicity, and complement activation (9). Several case studies have shown successful use of rituximab for inflammatory pseudotumors in the jaw (8) and temporal bone (9) in addition to IgG4 related disease in the orbit (10)(11)(13)(14) and temporal bone (15). No reports to date have used rituximab for successful treatment of TFIL specifically.…”

Section: Discussionmentioning

confidence: 99%

“…Past treatments have included combinations of steroids, surgical resection, radiation, and/or immunomodulators. Treatment with immunomodulators have included cyclophosphamide, mycophenolate mofetil, methotrexate, alpha interferon, azathioprine, and rituximab (1,2,4,(8)(9)(10)(11)(12)(13). Complete resection is preferred; however, tumor location may prevent a safe and tolerable approach (2).…”

Section: Introductionmentioning

confidence: 99%

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Tumefactive fibroinflammatory lesion successfully treated with Rituximab

Bishop

Bryan

Savage

et al. 2019

IRDR

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Tumefactive fibroinflammatory lesion successfully treated with Rituximab

Bishop

Bryan

Savage

et al. 2019

IRDR

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“…There have been a total of 36 reports describing 121 patients who received treatment with RTX for non-infectious scleritis [ 11 , 12 , 24 , 37 – 40 , 46 – 65 , 66 , 67 – 70 , 71 , 72 , 73 , 74 ]. These patients possessed a male to female ratio of 0.56 to 1 and a mean age of 48.5 ± 16.6 years (range = 16.0 to 81.0 years; median = 52.0 years) when first treated with RTX.…”

Section: Resultsmentioning

confidence: 99%

Rituximab for non-infectious Uveitis and Scleritis

Cunningham

2021

J Ophthal Inflamm Infect

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Purpose To provide a comprehensive review of rituximab use for the treatment of non-infectious uveitis and scleritis. Methods Review of literature through December 2020. Results Individual data was available for 229 patients with refractory non-infectious uveitis (n = 108) or scleritis (n = 121) who received treatment with rituximab (RTX). Rituximab was generally utilized as third-line or later treatment (uveitis: 67/90, 74.4%; scleritis: 90/96, 93.8%) at a mean of 33.5 months following the diagnosis of uveitis (range = 0 to 168.0 months; median = 24.0 months) and 39.4 months after diagnosis of scleritis (range = 1.0 to 168.0 months; median = 21.0 months). Patients with non-infectious uveitis and scleritis either received prior treatment with corticosteroids only (uveitis: 18/90, 20%; scleritis: 4/94, 4.3%), or with one (uveitis: 19/90, 21.1%; scleritis: 30/94, 31.9%), two (uveitis: 11/90, 12.2%; scleritis 27/94, 28.7%), or three or more (uveitis: 37/90, 41.1%; scleritis: 31/94, 33.0%) corticosteroid-sparing immunosuppressive agents with or without corticosteroids before initiation of RTX treatment. The rheumatologic protocol (two infusions of 1 gram of RTX separated by 14 days) was utilized most frequently (uveitis: 45/87, 51.7%; scleritis: 87/114, 76.3%), followed by the Foster protocol (eight weekly infusions of 375 mg/m2 RTX; uveitis: 18/87, 20.7%; scleritis: 10/114, 8.8%), and the oncologic protocol (four weekly infusions of 375 mg/m2 RTX; uveitis: 5/87, 5.7%; scleritis: 6/114, 5.3%). Various other off-label regimens were used infrequently (uveitis: 19/87, 21.8%; scleritis 11/114, 9.6%). Rituximab treatments resulted in a positive therapeutic response for the majority of patients with non-infectious uveitis (81/97, 83.5%). Commonly treated uveitic diagnoses included non-paraneoplastic autoimmune retinopathy (30/107, 28.0%), juvenile idiopathic arthritis (21/107, 19.6%), Vogt-Koyanagi-Harada disease (12/107, 11.2%), and Behçet disease (11/107, 10.3%). Cases of non-infectious scleritis were most commonly attributed to granulomatosis with polyangiitis (75/121, 62.0%) and rheumatoid arthritis (15/121, 12.4%), and showed an even greater rate of positive therapeutic response (112/120, 93.3%) following RTX treatment. No side effects were reported in 76.3% (74/97) of uveitis and 85.5% (71/83) scleritis cases. Of those cases associated with RTX-induced adverse events, the most common were infusion reactions of various severity (11/35, 31.4%). Conclusions Overall, RTX appeared to be both effective and well-tolerated as second or third-line therapy for patients with non-infectious uveitis and scleritis.

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“…The authors described three patients refractory or intolerant to conventional immunosuppressants or TNF inhibitors, suggesting a special consideration for RTX in inflammatory eye diseases secondary to RA or other rheumatologic disorders [62]. Other interesting case reports and small case series have also been reported [19,[63][64][65][66][67][68][69][70][71][72]. RTX yielded a rapid benefit in an interesting case of severe and refractory scleritis associated with IgG4-related disease and initially misdiagnosed as idiopathic scleritis, advocating for a potential role of CD20 blockade as a therapeutic option in such cases [73].…”

Section: Rituximabmentioning

confidence: 95%

Biologic Therapies and Small Molecules for the Management of Non-Infectious Scleritis: A Narrative Review

et al. 2021

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Scleritis refers to a wide spectrum of ocular conditions ranging from mild to sight-threatening scleral inflammation that may compromise visual function and threaten the anatomical integrity of the ocular globe. Most aggressive forms like necrotizing or posterior scleritis are often difficult-to-treat cases, refractory to conventional treatment. The association with systemic diseases, namely rheumatoid arthritis, Sjögren syndrome, granulomatosis with polyangiitis, and relapsing polychondritis, may have prognostic implications as well. A better understanding of the pathogenesis of ocular inflammatory diseases have paved the way to more effective and targeted treatment approaches. In this regard, a growing body of evidence supports the potential role of biologic agents in the management of non-infectious scleral inflammation, either idiopathic or in a background of immune-mediated systemic disorders. Biologic agents such as anti-tumor necrosis factor agents, interleukin-1 and interleukin-6 inhibitors as well as CD20 blockade have displayed promising results. More specifically, several studies have reported their ability to control scleral inflammation, reduce the overall scleritis relapses, and allow a glucocorticoid-sparing effect while being generally well tolerated. Anecdotal reports have also been described with other biologic agents including abatacept, ustekinumab, daclizumab, and alemtuzumab as well as targeted small molecules such as tofacitinib. Further studies are warranted to fully elucidate the role of biologic agents in non-infectious scleritis and investigate specific areas with the aim to administer treatments in the context of personalized medicine. This review summarizes the available data regarding clinical trials, small pilot studies, and real-life experience of the last two decades reporting the use of biologic agents in the management of non-infectious scleritis.

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Idiopathic Orbital Inflammation Associated With Necrotizing Scleritis and Temporal Bone Inflammation

Cited by 7 publications

References 8 publications

Tumefactive fibroinflammatory lesion successfully treated with Rituximab

Tumefactive fibroinflammatory lesion successfully treated with Rituximab

Rituximab for non-infectious Uveitis and Scleritis

Biologic Therapies and Small Molecules for the Management of Non-Infectious Scleritis: A Narrative Review

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