A 51-Year Old Woman with Schnitzler Syndrome Treated with Canakinumab

Abstract

Schnitzler syndrome is a rare auto-inflammatory disorder characterized by urticarial skin rash, a monoclonal IgM component and at least two of the following findings: periodic fever, arthralgias or arthritis, bone pain, palpable lymph nodes, liver or spleen enlargement, elevated erythrocyte sedimentation rate, leukocytosis, and abnormal findings on bone morphologic investigations. We present the first case of Schnitzler syndrome successfully treated with Canakinumab, a novel anti-IL-1-beta monoclonal antibody.