Purpose: To review adult Still’s disease (ASD), especially therapeutic strategies by biologics.
Methods: Literature about the use of biologics in ASD by the PubMed search were found.
Results: ASD is an inflammatory disorder of unknown cause, characterized by a spiking fever, joint pains and an evanescent rash. Proinflammatory cytokine and chemokines, such as IL-6, IL-18, TNF-α and CXCL10 (IP-10) are involved in the pathophysiology of ASD. Preliminary classification criteria for ASD by Yamaguchi et al. is used all over the world. There have been thirteen reports in the literature on several intractable patients at onset age 16 years or older with ASD treated with biologics including anakinra, tocilizumab, infliximab, or etanercept. These therapies were effective for most patients. Adverse events such as infection and injection-site reaction were reported. Most of studies were case series. A diverse definition of treatment-resistant cases was adopted.
Conclusions: A nation-wide registry or a prospective, double blind, randomized study would be desirable in future.