Treatment of adult-onset Still’s disease: a review

Abstract

Adult-onset Still’s disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. The former classification, based on the disease course, seems to be quite dated. Indeed, there is accumulating evidence that AOSD can be divided into two distinct phenotypes based on cytokine profile, clinical presentation, and outcome, ie, a “systemic” pattern and an “articular” pattern. The first part of this review deals with the treatments that are currently available for AOSD. We then present the different strategies based on the characteristics of the disease according to clinical presentation. To do so, we focus on the two subsets of the disease. Finally, we discuss the management of life-threatening complications of AOSD, along with the therapeutic options during pregnancy.

Keywords:

• adult-onset Still’s disease
• treatment
• anakinra
• tocilizumab
• canakinumab

Acknowledgments

YJ acknowledges the Foundation for the Development of Internal Medicine in Europe, the Société Nationale Française de Médecine Interne, Groupama Fundation, and Genzyme for their help in funding his PhD research project. YJ is supported by a “poste d’accueil” at INSERM.

Disclosure

The authors report no conflicts of interest in this work.