Successful anakinra for refractory Schnitzler syndrome

Schnitzler's syndrome is a rather rare disease which may appear in a rheumatologist's office because patients often report rheumatic symptoms with joint, bone and muscle pain. However, it is characterized by chronic urticaria, recurrent fever, liver and spleen enlargement, osteosclerosis, and lymphadenopathy, in conjunction with a serum IgM M component. A patient who had been treated with relatively high doses of corticosteroids for 10 years with insufficient response was treated with the IL-1 receptor antagonist anakinra, this led to a complete resolution of symptoms.