Abstract:BackgroundHyper-IgD syndrome/mevalonate kinase deficiency (HIDS/MKD) is a rare autoinflammatory condition caused by a defect in the gene coding for mevalonate kinase. This periodic fever syndrome is characterized by severe systemic and organ inflammation. Treatment with interleukin-1β inhibitor canakinumab (CAN), approved and applied for treatment of HIDS/MKD patients since 2017 [1], resulted in rapid remission of symptoms and normalization of laboratory parameters in most patients in clinical trials [2] as we… Show more
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