Schnitzler Syndrome

Abstract

The Schnitzler syndrome is a chronic non-pruritic urticaria within a monoclonal IgM gammopathy, associated with recurrent fever, arthralgia, weight loss and lymphadenopathy. Leucocytosis and altered inflammatory markers could be observed. Thus, the disease is now considered a late-onset acquired autoinflammatory syndrome. Diagnosis relies on a combination of clinical, biological and radiological findings as well as on exclusion of other causes. Currently, there is no approved treatment for the Schnitzler syndrome. Interleukin-1 blocking agents seem to be the most effective therapies, even though only on the inflammation-linked symptoms, not on the monoclonal component.