Efficacy and safety of canakinumab treatment in schnitzler syndrome: A systematic literature review.

BACKGROUND

Schnitzler syndrome is a rare autoinflammatory disorder characterized by chronic urticarial rash and a monoclonal gammopathy, accompanied by intermittent fever, bone pain, and arthralgia or arthritis. Canakinumab is a fully human monoclonal anti-interleukin-1β (IL-1β) antibody proven to be effective in IL-1 driven autoinflammatory disorders.

METHODS

We systematically searched PubMed and Embase to include all types of studies on canakinumab treatment in Schnitzler syndrome published until March 16, 2020.

RESULTS

Since 2011, 7 publications have been reported on canakinumab treatment in 34 patients with Schnitzler syndrome. The cumulative follow-up was 253 months, and 5 studies had a follow-up duration of 12 months or more. A complete response during treatment was reported in 58.6% of patients; all other patients had a partial response. Two hundred and seven adverse events were reported in 23 patients. Infection (n = 79) was the most common adverse event. One patient died from sepsis due to atypical mycobacterial infection.

CONCLUSION

Based on the results of the current systematic review, canakinumab is an effective long-term treatment with a favorable safety profile in patients with Schnitzler syndrome.