Synopsis

Schnitzler syndrome

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Synopsis

Schnitzler syndrome is a rare disabling disorder characterised by a chronic urticarial rash and a monoclonal IgM gammopathy, accompanied by at least two of the following features: intermittent unexplained fever, arthralgia or arthritis, bone pain, lymphadenopathy, hepato- or splenomegaly, leukocytosis and an elevated ESR, and abnormal findings on bone morphologic investigations. In 1972, Schnitzler was the first to describe the syndrome, and to date, about 94 cases have been reported. In most cases, the syndrome follows a chronic, benign course, but at least 10% will develop a lymphoproliferative disorder in the long term. The pathophysiology is still unknown. Treatment options include steroids. Recently, monotherapy with interleukin-1 inhibitor Anakinra was discovered to be very effective in treating symptoms of Schnitzler syndrome.

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Last update/check: June 30, 2007