@Article{ecn.2009.0164,
AUTHOR = {Paola Migliorini, Isabella Del Corso, Cristina Tommasi, Diana Boraschi},
TITLE = {Free circulating interleukin-18 is increased in Schnitzler syndrome: a new autoinflammatory disease?},
JOURNAL = {European Cytokine Network},
VOLUME = {20},
YEAR = {2009},
NUMBER = {3},
PAGES = {108--111},
URL = {http://www.techscience.com/ECN/v20n3/65895},
ISSN = {1952-4005},
ABSTRACT = {Schnitzler syndrome is a rare disease characterised by chronic urticaria and arthralgia. The
recent evidence that the IL-1 receptor antagonist IL-1Ra could induce rapid and complete remission of Schnit-zler
symptoms has pointed to IL-1 as a major pathological factor in this disease. To examine the possibility
that Schnitzler syndrome may be considered to be an autoinflammatory disease, in this study we measured the
serum levels of IL-18, another cytokine of the IL-1 family that is cleaved by caspase-1, in two recently diag-nosed
Schnitzler patients before and after treatment with IL-1Ra. In parallel, mRNA expression of IL-1 family
cytokines and caspase-1 were assessed in isolated blood monocytes. Treatment with IL-1Ra significantly inhib-ited
IL-1β gene expression, indicating that IL-1β activity in Schnitzler syndrome is central to IL-1β gene upre-gulation
in a type of auto-amplification loop. While no IL-1β was detected in serum, free circulating IL-18 was
increased in patients with Schnitzler syndrome, despite low IL-18 gene expression in monocytes. This suggests
constitutive activation of the IL-1β/IL-18-producing inflammasome, and supports the hypothesis that
Schnitzler’s syndrome is a new autoinflammatory disease.},
DOI = {10.1684/ecn.2009.0164}
}