Canakinumab injection for the treatment of active Still’s disease, including adult-onset Still’s disease

ABSTRACT

Introduction

Still’s disease is a systemic, non-monogenic autoinflammatory condition affecting both children and adolescents (systemic juvenile idiopathic arthritis, sJIA) and adults (adult-onset Still’s disease, AOSD). Its clinical spectrum ranges from mild forms to life-threatening cases. Glucocorticoids represent the first-line therapy, but their chronic use is burdened with significant side effects. Hence, add-on therapy with disease modifying anti-rheumatic drugs and biologic anti-cytokine agents is frequently required, especially in patients with severe and recalcitrant clinical phenotypes. Among the targetable cytokines with a primary role in the pathogenesis of Still’s disease, interleukin-1 has a leading position.

Areas covered

This review presents the available controlled evidence and observational studies regarding the efficacy and safety of canakinumab, a monoclonal antibody targeting interleukin-1β, in the treatment of Still’s disease.

Expert opinion

Controlled studies fully support the clinical efficacy of canakinumab in the treatment of patients affected by sJIA. Conversely, strong evidences are still lacking in AOSD patients; nevertheless, its use in the adult population is legitimated by an increasing number of cases series and case reports and by preclinical data linking the pathogenesis of these two diseases. In addition, canakinumab has an excellent safety profile. Its role in preventing and treating macrophage activation syndrome is still debated.

KEYWORDS:

• Still’s disease
• systemic juvenile idiopathic arthritis
• adult onset still’s disease
• aosd
• canakinumab
• interleukin-1
• macrophage activation syndrome

Article highlights

• Still’s disease is a disease continuum comprising two main entities: systemic juvenile idiopathic arthritis (sJIA) and adult onset Still’s disease (AOSD).

• Interleukin-1 has a pivotal role in both sJIA and AOSD pathogenesis.

• Canakinumab is a monoclonal antibody specifically targeting IL-1β.

• Controlled studies have demonstrated the efficacy of canakinumab in treating children and adolescents affected by sJIA.

• The use of canakinumab in AOSD mainly relies on case reports and case series.

• No significant adverse events have been described with the use of canakinumab neither in children and young adolescents nor in adults.

• Available evidences suggest that IL-inhibition through canakinumab is not sufficient to reduce the risk of developing macrophages activation syndrome.

This box summarizes key points contained in the article.

Box 1. Drug summary box

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Declaration of interest

L Dagna and C Campochiaro receive consulting fees from SOBI, Novartis. G Cavalli receives consulting fees from SOBI, Novartis, Cerecor. G De Luca receives consulting fees from Novartis. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.