Schnitzler syndrome refractory to anakinra: successful treatment with canakinumab

Abstract

Schnitzler syndrome (SchS) is a rare autoimmune and inflammatory disease mediated by interleukin-1 beta (IL-1β). Recurrent monoclonal gammopathy and chronic urticarial rash are the symptoms required for diagnosis according to the Strasbourg criteria. The low prevalence of this syndrome (around 300 cases have been reported) and confusion with other inflammatory disorders may delay the diagnosis for up to 5 years. Although the most effective treatment for SchS is anakinra, some patients do not respond to this treatment. We report a case of SchS in a 64-year-old woman with multiple episodes of fever, severe rash, erythema, arthralgia and dyspnea. The patient was successfully treated with canakinumab after anakinra intolerance and failure of colchicine, prednisone, methotrexate and dapsone. After the first dose of canakinumab the skin wounds rapidly improved and the patient did not require any concomitant treatments. The cause of SchS is still unknown and a differential diagnosis is recommended, especially with adult-onset Still´s disease due to their similar symptoms. Canakinumab, a specific anti-IL-1β antibody, blocks its binding to receptors, thereby preventing IL-1β-induced gene activation and production of inflammatory mediators. Canakinumab has proven to be an effective drug in SchS, providing an alternative to anakinra.

Keywords:

• Anakinra
• autoinflammatory
• canakinumab
• Schnitzler syndrome (SchS)

Authors’ contributions

MDF and NST were involved in the diagnosis and management of the patient. SGM reviewed the literature. ALSM drafted the original manuscript and coordinated the study. PCR and BGD carried out the final editing of the manuscript. All authors read and approved the final manuscript.

Compensation

The authors declare that no other person has made a substantial contribution to this manuscript. Nevertheless, we thank the patient for granting permission to publish this information.

Disclosure statement

The authors declare no potential conflicts of interest, including relevant financial interests, activities, relationships and affiliations with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript.

Patient consent for publication

The informed consent was obtained from the patient for publication of this case report.

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The authors declare that this paper is original and has not been previously published or is in the process of being reviewed by any other journal.

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This manuscript has been performed without receiving funding nor grants, consultancies, honoraria or payment, speakers’ bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending or issued.

Data availability statement

Data sharing not applicable to this article as no datasets were generated or analyzed during the current study.

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.