Recent advances in the treatment of adult-onset Still's disease

Abstract

Introduction: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder. The disease course can be stratified as monocyclic systemic, polycyclic systemic and chronic articular patterns. At present, there is no gold standard of treatment for AOSD. Recent insights into AOSD pathogenesis have led to major changes in treatment.

Areas covered: This paper focuses on current biological therapy for AOSD with different disease course.

Expert opinion: Traditional approach to AOSD treatment has been the use of nonsteroidal anti-inflammatory drugs (NSAIDs) usually in combination with corticosteroids. In patients with polycyclic systemic or chronic articular pattern, combination with disease-modifying antirheumatic drugs (DMARDs) has been shown to have increased efficacy. Given that proinflammatory cytokines are implicated in AOSD pathogenesis, biological therapy is a logical therapeutic approach for those with inadequate response to the first-line therapy. Response to anti-IL-1β agents, such as anakinra, was rapid and sustained in most patients with systemic pattern, but might be less effective in those with chronic arthritis. Anti-tumor necrosis factor-alpha (TNF-α) therapy has been shown to be effective in refractory AOSD patients, particularly in those with chronic arthritis. Anti-IL-6 agents appear to be effective in AOSD patients with polycyclic systemic or chronic articular pattern and may be a promising therapeutic choice for those refractory to anti-IL-1β or anti-TNF-α therapy.

Keywords::

• adult-onset Still's disease
• cytokines
• disease courses
• pathogenesis
• treatment

Notes

This box summarizes key points contained in the article.