Abstract

Schnitzler's syndrome (SchS) is a rare, disabling, autoinflammatory disorder characterized by recurrent urticarial rash and monoclonal IgM gammopathy. Interleukin-1 beta (IL-1β) plays an important role in the pathophysiology of SchS. Only anecdotal reports demonstrate the efficiency and safety of human monoclonal anti-human IL-1β antibody (canakinumab) use in SchS therapy. However, there are no generally accepted recommendations concerning the scheme (or frequency) of canakinumab use for this disease. Here, we report the effective long-term treatment of SchS in a 44-year-old male with a standard canakinumab dose (150 mg) but with an increased 4-month injection interval.

Highlights

  • Schnitzler’s syndrome (SchS) is a chronic, disabling, autoinflammatory disorder characterized by chronic urticarial rash and a monoclonal component [1]

  • We present a case of efficient and safe use of canakinumab with increased injection interval in a patient with SchS

  • SchS is a rare disease; since its first description in 1972, fewer than 300 cases have been reported in the literature [2, 4]. e pathogenesis of SchS is unknown. e excellent efficacy of sanakinumab, which selectively inhibits IL-1β, suggests a key role of this cytokine in the pathogenesis of SchS [6, 7, 9,10,11]

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Summary

Case Report

Increasing the Interval of Canakinumab Administration Effectively Supports the Remission of Schnitzler’s Syndrome. V.A. Nasonova Research Institute of Rheumatology, Russian Academy of Medical Sciences, Kashirskoye Shosse 34A, Moscow 115522, Russia. Received October 2017; Revised 7 March 2018; Accepted March 2018; Published 11 April 2018. Schnitzler’s syndrome (SchS) is a rare, disabling, autoinflammatory disorder characterized by recurrent urticarial rash and monoclonal IgM gammopathy. Anecdotal reports demonstrate the efficiency and safety of human monoclonal anti-human IL-1β antibody (canakinumab) use in SchS therapy. There are no generally accepted recommendations concerning the scheme (or frequency) of canakinumab use for this disease. We report the effective longterm treatment of SchS in a 44-year-old male with a standard canakinumab dose (150 mg) but with an increased 4-month injection interval

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