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Rheumatology Advance Access originally published online on August 20, 2009
Rheumatology 2009 48(10):1304-1308; doi:10.1093/rheumatology/kep222
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Dysfunctional inflammasome in Schnitzler's syndrome

Cinzia Pizzirani1,2,*, Simonetta Falzoni1,2,*, Marcello Govoni2,3, Renato La Corte2,3, Simona Donadei4, Francesco Di Virgilio1,2, Francesco Trotta2,3 and Andrea Lo Monaco2,3

1Department of Experimental and Diagnostic Medicine, Section of General Pathology, 2The Study of Inflammation (ICSI), 3Department of Experimental and Clinical Medicine, Section of Rheumatology, University of Ferrara, Ferrara and 4Amyloid Centre, Biotechnology Research Laboratories-Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

Correspondence to: Andrea Lo Monaco, Azienda Universitaria-Ospedaliera S. Anna, C.so della Giovecca n. 203, Ferrara 44100, Italy. E-mail: lmnndr{at}unife.it


  Abstract

Objective. IL-1β plays a key role in the pathogenesis of Schnitzler's syndrome (SS). We have investigated inflammasome activity in peripheral blood mononuclear cells (PBMCs) from a patient affected by a variant type of SS.

Methods. PBMCs were purified by Ficoll and examined for ability to secrete IL-1β and -18, expression and function of the P2X7 receptor and expression of apoptosis-associated speck-like protein containing a caspase recruitment domaine (ASC) and NOD-like receptor protein 3 (NLRP3) before and after the therapy with steroid. Furthermore, extracellular adenosine 5'-triphosphate (ATP) blood levels were determined by luciferase assay. Expression of inflammasome components was measured by real time PCR and western blotting.

Results. PBMCs of patient with SS showed a high, spontaneous and lipopolysaccharide-stimulated, IL-1β release but low response to stimulation with the P2X7 agonist benzoyl ATP. P2X7 expression was several fold increased, whereas ASC expression was dramatically decreased compared with PBMCs from healthy controls. NLRP3 expression was unchanged. Prednisone treatment induced remission of clinical symptoms and normalized IL-1β secretion and P2X7 and ASC expression.

Conclusion. These findings reveal the presence of an overall derangement of the inflammasome and IL-1β processing and release in SS.

KEY WORDS: Schnitzler's syndrome, Autoinflammatory diseases, Inflammasome, Interleukin-1β, P2X7 receptor

*Cinzia Pizzirani and Simonetta Falzoni equally contributed to this work.

Submitted 28 October 2008; revised version accepted 29 June 2009.
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