Jorch, S. K.; McNally, A.; Berger, P.; Wolf, J.; Kaiser, K.; Chetrusca Covash, A.; Robeck, S.; Pastau, I.; Fehler, O.; Jauch-Speer, S.-L.; Hermann, S.; Schäfers, M.; Van Gorp, H.; Kanneganti, A.; Dehoorne, J.; Haerynck, F.; Penco, F.; Gattorno, M.; Chae, J. J.; Kubes, P.; Lamkanfi, M.; Wullaert, A.; Sperandio, M.; Vogl, T.; Roth, J.; Austermann, J.
Familial Mediterranean Fever (FMF), caused by mutations in the pyrin encoding MEFV gene, is characterized by uncontrolled caspase-1 activation and IL-1ß secretion. A similar mechanism drives inflammation in cryopyrin-associated periodic fever syndrome (CAPS) caused by mutations in NLRP3. CAPS and FMF, however, result in largely different clinical manifestations pointing to additional, autoinflammatory pathways involved in FMF. Another hallmark of FMF is the extraordinarily high expression of S100A8 and S100A9. These alarmins are ligands of Toll-like receptor 4 and amplifiers of inflammation. However, the relevance of this inflammatory pathway for the pathogenesis of FMF is unknown.