Elevated serum levels of checkpoint molecules in patients with adult Still’s disease

Fujita, Yuya; Asano, Tomohiko; Matsumoto, Haruki; Matsuoka, Naoki; Temmoku, Jumpei; Sato, Shuzo; Furuya, Makiko Yashiro; Suzuki, Eiji; Watanabe, Hiroshi; Koga, Tomohiro; Kawakami, Atsushi; Migita, Kiyoshi

doi:10.21203/rs.3.rs-26391/v2

Cited by 2 publications

(2 citation statements)

References 29 publications

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“…Thus, various danger signals seem to be involved in the pathogenesis of AOSD. T cell immunoglobulin and mucin-containing-molecule-3 (TIM-3), an immune checkpoint molecule, was recently shown to control not only adaptive immunity but also innate immunity (50), and we reported that TIM-3 is significantly elevated in patients with AOSD (51). Nonetheless, due to the diversity of this immune checkpoint molecule's functions, further research is necessary to elucidate its role in AOSD.…”

Section: Pathogenesismentioning

confidence: 98%

The Expanding Spectrum of Autoinflammatory Diseases

et al. 2023

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Autoinflammatory diseases are systemic disorders caused by genetic or acquired abnormalities in certain signaling pathways of the innate immune system. Dysregulated activation of the inflammasome, i.e. molecular platforms responsible for the activation of caspase-1 and production of interleukin-1β, causes autoinflammation. Familial Mediterranean fever (FMF), the most common genetic autoinflammatory disease, is characterized by a periodic fever and serositis. The complex and heterogeneous genetic background of Japanese FMF patients, accompanied by potential overlap with other rheumatic diseases, suggests crosstalk between genetic and environmental factors. Recently, FMF has been recognized as being part of a spectrum of autoinflammatory syndromes named pyrin-associated autoinflammatory diseases. The discovery of a new monogenic autoinflammatory disease, A20 haploinsufficiency, may provide novel insights into early-onset Behçet's-like diseases. In contrast, adult-onset Still's disease and Schnitzler's syndrome are acquired autoinflammatory diseases without a monogenic abnormality. Although the concept of autoinflammatory diseases originally applied to monogenic hereditary recurrent fevers, it has been expanded to include non-genetic complex autoinflammatory diseases. Information concerning monogenic autoinflammatory diseases may prove useful for elucidating the molecular mechanisms underlying non-genetic autoinflammatory diseases.

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Section: Pathogenesismentioning

confidence: 98%

The Expanding Spectrum of Autoinflammatory Diseases

et al. 2023

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“…These drugs affect membrane stability and alter transcriptional activity, leading to the inhibition of cytokine production and modulation of several co-stimulatory molecules (Schrezenmeier and Dorner, 2020). The anti-inflammatory or immunomodulatory effect may originate from a significant decrease in the production of cytokines, particularly pro-inflammatory factors including interleukin (IL)-6 and IL-1β (Ornstein and Sperber, 1996;Fujita et al, 2019).…”

Section: Mechanisms Of Therapeutic Actionmentioning

confidence: 99%

Hydroxychloroquine-induced Retinal Toxicity

2023

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Long-term use of hydroxychloroquine can cause retinopathy, which may result in severe and progressive visual loss. In the past decade, hydroxychloroquine use has markedly increased and modern retinal imaging techniques have enabled the detection of early, pre-symptomatic disease. As a consequence, the prevalence of retinal toxicity in long-term hydroxychloroquine users is known to be higher than was previously estimated. The pathophysiology of the retinopathy is incompletely characterised, although significant advances have been made in understanding the disease from clinical imaging studies. Hydroxychloroquine retinopathy elicits sufficient public health concern to justify the implementation of retinopathy screening programs for patients at risk. Here, we describe the historical background of hydroxychloroquine retinopathy and summarize its current understanding. We review the utility and limitations of each of the mainstream diagnostic tests used to detect hydroxychloroquine retinopathy. The key considerations towards a consensus on the definition of hydroxychloroquine retinopathy are outlined in the context of what is known of the natural history of the disease. We compare the current screening recommendations for hydroxychloroquine retinopathy, identifying where additional evidence is required, and the management of proven cases of toxicity. Finally, we highlight the areas for further investigation, which may further reduce the risk of visual loss in hydroxychloroquine users.

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Elevated serum levels of checkpoint molecules in patients with adult Still’s disease

Cited by 2 publications

References 29 publications

The Expanding Spectrum of Autoinflammatory Diseases

The Expanding Spectrum of Autoinflammatory Diseases

Hydroxychloroquine-induced Retinal Toxicity

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