PReS-FINAL-2330: Canakinumab Treatment in Patients with HIDS

Hyper-IgD and periodic-fever syndrome (HIDS) is a recessively inherited-autoinflammatory condition caused by mevalonate-kinase mutations. It is characterized by early-onset (<1 year of age), 4-6 days-long acute inflammatory episodes that typically recur every 4-6 weeks. The main features during episodes include fever, lymphadenopathies, rash, headache, abdominal pain, diarrhea, and a marked acute phase reaction[1,2]. Previous case reports suggested IL-blockade as a potential therapy. Canakinumab (CAN) is a fully-human, selective anti-IL-1β monoclonal-antibody (MoAb). Preliminary clinical and pharmacokinetics (PK) data of CAN-therapy in active HIDS patients is presented.