PReS-FINAL-2240: Serum amyloid protein a concentration in CAPS patients treated with anti IL1Β

Introduction Although all patients with chronic inflammatory conditions are at risk for developing type AA amyloidosis, the incidence varies widely between the different Autoinflammatory syndromes. The reported incidence is about 35% of patients with CAPS (cryopyrin-associated periodic syndrome) which comprises familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome and CINCA (chronic infantile neurological cutaneous, articular inflammatory syndrome). CAPS is associated with mutations in NLRP3/CIAS1 on chromosome 1q44, found only in about 50% of patients. Previous studies have demonstrated that IL-1b inhibitors are able to induce complete remission of clinical manifestations and suppression of markers of inflammation in the majority of patients.