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Autoinflammatory Pathogenesis and Targeted Therapy for Adult-Onset Still’s Disease

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Abstract

Adult-onset Still’s disease (AOSD) is a rare multisystem autoinflammatory disorder of unknown etiology. AOSD is generally characterized by high spiking fever, arthralgia or arthritis, skin rash, leukocytosis, and hyperferritinemia. Traditionally, AOSD has been treated with non-steroidal anti-inflammatory drugs, corticosteroids, and immunosuppressants. An increasing number of studies have shown that proinflammatory cytokines, such as interleukin-1β, -18, -6, and tumor necrosis factor-α, play key roles in AOSD and may serve as therapeutic targets. In the current review, we provided insights into the roles of these cytokines in the pathogenesis of AOSD and also provided a commentary on the clinical studies of biologic therapy against AOSD.

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Abbreviations

AOSD:

adult-onset Still’s disease

DAMPs:

danger-associated molecular patterns

DMARDs:

disease-modifying antirheumatic drugs

HLA:

human leukocyte antigen

IL:

interleukin

IL-18BP:

IL-18 binding protein

NLRP:

nucleotide-binding oligomerization-domain-like receptor family, pyrin domain containing

PAMPs:

pathogen-associated molecular patterns

PBMCs:

peripheral blood mononuclear cells

sIL-6R:

soluble form of IL-6 receptor

SJIA:

systemic juvenile idiopathic arthritis

STAT:

signal transducer and activator of transcription

TLRs:

Toll-like receptors

TNF:

tumor necrosis factor

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Funding

This work was supported by the National Natural Science Foundation of China grants (#81571969 to JQ and 81673045 to HF).

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  1. Department of Dermatology, the First Affiliated Hospital, College of Medicine, Zhejiang University, No. 79 Qingchun Road, Hangzhou, 310003, China

    Sheng Li, Siting Zheng, Shunli Tang, Yunlei Pan, Shan Zhang, Hong Fang & Jianjun Qiao

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  1. Sheng Li
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  2. Siting Zheng
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Li, S., Zheng, S., Tang, S. et al. Autoinflammatory Pathogenesis and Targeted Therapy for Adult-Onset Still’s Disease. Clinic Rev Allerg Immunol 58, 71–81 (2020). https://doi.org/10.1007/s12016-019-08747-8

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