Long Term Efficacy of BID Administration of Anakinra and Canakinumab in Two Refractory Cases of Schnitzler Syndrome

Case Series

Volume 3, Issue 1 (January Issue)

Long Term Efficacy of BID Administration of Anakinra and Canakinumab in Two Refractory Cases of Schnitzler Syndrome

Annacarla Finucci^1*, Ditto Maria Chiara¹, Parisi Simone¹, Borrelli Richard¹, Piercarlo Sarzi Puttini², Renato Alberto Sinico³ and Fusaro Enrico¹

¹Rheumatology Unit, AOU Citta della Salute e della Scienza, Turin, Italy
²Rheumatology Unit, ASST Fatebenefratelli-Sacco, Milan, Italy
³Medicine and Surgery Department, Milano Bicocca University, Monza, Italy

^*Corresponding author: Annacarla Finucci, Rheumatology Unit, AOU Citta della Salute e della Scienza, Corso Bramante 88, 10126, Turin, Italy, Tel: +390116335448; E-mail: annacarla.finucci@gmail.com

Received: September 15, 2020; Accepted: October 12, 2020; Published: November 03, 2020

Citation: Annacarla Finucci, Ditto Maria Chiara, Parisi Simone, et al. Long Term Efficacy of BID Administration of Anakinra and Canakinumab in Two Refractory Cases of Schnitzler Syndrome. Clin Image Case Rep J. 2021; 3(1): 129.

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Long Term Efficacy of BID Administration of Anakinra and Canakinumab in Two Refractory Cases of Schnitzler Syndrome

Abstract

Introduction: Schnitzler Syndrome (SS) is a rare, systemic acquired autoinflammatory syndrome. Its diagnosis is challenging due to confounding factors with systemic autoimmune diseases. In 2013 Strasbourg criteria for SS were validated. Until 2005, no effective therapy was available. The efficacy of IL-1 blockers is supported by several case reports as well as by some clinical studies; however, to date, they are not approved for SS.

Cases: Here we describe two cases of SS who did not respond to daily anakinra, efficaciously treated with anakinra bid and canakinumab, respectively. The two cases also evidence how SS diagnosis can be difficult, mimicking different pathologies.

Conclusion: SS is a complex disease, in which diagnostic process could frequently belong and not linear. A better knowledge of the disease and the possibility to use IL1 blockers modified disease outcome, leading to the achievement of persistent clinical remission.

Keywords: Schnitzler syndrome; Anakinra; Canakinumab