Modern look at Schnitzler syndrome

Schnitzler syndrome (SS) is an extremely rare acquired systemic disease that shares many similarities with various hereditary autoinflammatory syndromes. It presents as chronic non-pruritic urticarial rash, monoclonal gammopathy, and systemic symptoms, such as recurrent fever, arthralgia, myalgia, bone pain, bone lesions, and enlargement of the spleen and liver. The specific feature associated with SS is its spectacular response to treatment using anti-interleukin-1 (anti-IL-1) agents, such as anakinra or canakinumab. If it remains untreated, the disease can have a devastating effect on the patient's quality of life as well as increased mortality due to systemic complications. Herein, we will summarize the most recent findings in the pathogenesis, diagnosis, and management of SS.