SCHNITZLER SYNDROME: MORE THAN CHRONIC URTICARIA

Abstract

Schnitzler syndrome is a rare acquired autoinflammatory disorder characterized by urticarial rash, fever, bone and joint pain, and lymphadenopathy. It is associated with monoclonal gammopathy and increased inflammatory parameters. The disease usually begins in the late 50s and is more common in men. The syndrome is diagnosed according to Strasbourg diagnostic criteria after excluding possible differential diagnoses. The disease lasts a lifetime. About 15–20% of patients develop lymphoproliferative disease and, in rare cases, AA amyloidosis. The most effective treatment is interleukin (IL) 1 antagonist. The article reviews the pathogenesis, clinical presentation, diagnosis and treatment of Schnitzler syndrome and presents clinical cases.