Scleritis associated with relapsing polychondritis

Maza, Maite Sainz de la; Molina, Nicolas; González-González, Luis; Doctor, Priyanka; Tauber, Joseph; Foster, C. Stephen

doi:10.1136/bjophthalmol-2015-306902

Cited by 37 publications

(15 citation statements)

References 20 publications

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“…Scleritis may be the initial manifestation of RP and has some typical characteristics when connected with RP. Sainz-de-la-Maza et al [22], in a retrospective analysis of 13 RP patients diagnosed with scleritis, compared 113 patients with other autoimmune diseases and showed that scleritis in RP patients was more often bilateral ( p = 0.001), necrotising (23.1%; p = 0.02), recurrent ( p = 0.001), and associated with decreased vision ( p = 0.012). A significant proportion of RP patients from that study (9/13, 69.2%) had one or more other autoimmune conditions, which on average preceded RP by nine years (between two and 21 years).…”

Section: Discussionmentioning

confidence: 99%

Relapsing polychondritis – analysis of symptoms and criteria

Maciążek-Chyra¹,

Szmyrka²,

Skoczyńska³

et al. 2019

Reumatologia

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Section: Discussionmentioning

confidence: 99%

Relapsing polychondritis – analysis of symptoms and criteria

Maciążek-Chyra¹,

Szmyrka²,

Skoczyńska³

et al. 2019

Reumatologia

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“…Moreover, they allowed a GC-sparing effect and were associated with a good safety profile [ 11 ]. Successful results with TNFα inhibitors have also been reported in three patients affected by relapsing polychondritis (RP) [ 20 ]. Table 1 shows the retrieved studies regarding TNF inhibitors employed in the management of non-infectious scleritis and some of their respective findings.…”

Section: Tumor Necrosis Factor Inhibitorsmentioning

confidence: 99%

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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“…Scleritis/episcleritis is the most common ocular complication associated with RPC (Table 3) [10,[26][27][28][29][30][31][32][33][34]. Most cases of scleritis occur bilaterally.…”

Section: Scleramentioning

confidence: 99%

“…Most cases of scleritis occur bilaterally. Several case series revealed that diffuse anterior scleritis was the most common (Figure 1), but nodular (Figure 2A) or necrotizing anterior scleritis and posterior scleritis (Figure 2B) were also observed (Table 4) [32][33][34][35]. Sainz-de-la-Maza et al compared patients with scleritis associated with RPC and other systemic immune-mediated diseases and showed that it is more often bilateral, recurrent, necrotizing, and associated with visual disturbance than scleritis associated with other systemic immune-mediated diseases [32].…”

Section: Scleramentioning

confidence: 99%

Ocular Involvement in Relapsing Polychondritis

Fukuda

Mizobuchi

Nakajima

et al. 2021

JCM

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Relapsing polychondritis (RPC) is a rare systemic immune-mediated disease characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body. Auricular, nasal, tracheal, and articular chondritis and arthritis are common systemic symptoms in patients with RPC. Ocular tissues are also targets of inflammation in RPC, and a variety of ocular symptoms are observed in approximately half of the patients with RPC. Scleritis/episcleritis, uveitis, and conjunctivitis are common symptoms associated with RPC. Less frequently, keratitis, retinopathy, optic neuropathy, muscle palsy, and orbital inflammation are also observed. Ocular inflammation could also be the first manifestation of RPC. Although RPC is a potentially fatal and sight-threatening disease, the rarity of the disease and its protean clinical presentation may lead to delayed diagnosis or misdiagnosis. Given the high prevalence of ocular involvement in RPC, to avoid misdiagnosis, physicians should be suspicious of RPC when they see patients with recurrent ocular inflammatory conditions and various systemic symptoms. In this article, we provide a comprehensive review of ocular manifestations associated with RPC.

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Scleritis associated with relapsing polychondritis

Cited by 37 publications

References 20 publications

Relapsing polychondritis – analysis of symptoms and criteria

Relapsing polychondritis – analysis of symptoms and criteria

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

Ocular Involvement in Relapsing Polychondritis

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