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Article Details
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 Finucci1*, 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.

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