Current and emerging biological therapy in adult-onset Still's disease

Yuning Ma; Jianfen Meng; Jinchao Jia; Mengyan Wang; Jialin Teng; Dehao Zhu; Chengde Yang; Qiongyi Hu

doi:10.1093/rheumatology/keab485

Current and emerging biological therapy in adult-onset Still's disease

Rheumatology (Oxford). 2021 Sep 1;60(9):3986-4000. doi: 10.1093/rheumatology/keab485.

Authors

Yuning Ma¹, Jianfen Meng^{1

2}, Jinchao Jia¹, Mengyan Wang¹, Jialin Teng¹, Dehao Zhu¹, Chengde Yang¹, Qiongyi Hu¹

Affiliations

¹ Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai.
² Department of Rheumatology and Immunology, The Fourth Affiliated Hospital of Nantong University, The First People's Hospital of Yancheng, Yancheng, China.

Abstract

Adult-onset Still's disease (AOSD) is a rare, but characteristic non-familial, multi-genic systemic auto-inflammatory disorder, characterized by high spiking fever, salmon-like evanescent skin rash, polyarthritis, sore throat, hyperferritinemia and leucocytosis. The hallmark of AOSD is a cytokine storm triggered by dysregulation of inflammation. Nowadays, with advances in anti-cytokine biologic agents, the treatment of AOSD is no longer limited to NSAIDs, glucocorticoids or conventional synthetic DMARDs. In this review, we focussed on the roles of these cytokines in the pathogenesis of AOSD and summarized the current and emerging biological therapy.

Keywords: adult-onset Still’s disease; biological therapy; cytokine storm.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Biological Therapy / methods*
Cytokine Release Syndrome / immunology
Cytokine Release Syndrome / therapy
Humans
Still's Disease, Adult-Onset / immunology
Still's Disease, Adult-Onset / therapy*