Schnitzler syndrome.
Schnitzler syndrome is characterized by monoclonal IgM gammopathy, urticaria, recurrent fever, evidence of inflammation, bone pain, and arthralgia, occasionally in combination with lymphadenopathy and/or hepatosplenomegaly. Only about 80 cases have been reported to date. Development of a hematological malignancy is the main complication. Recent reports of remissions induced by IL-1 receptor antagonist therapy shed new light on the pathophysiology of the disease.
Soubrier
Martin
M
Gabriel Montpied Teaching Hospital, Service de Rhumatologie, 58 rue Montalembert, BP 69, 63003 Clermont-Ferrand Cedex 1, France. msoubrier@chu-clermontferrand.fr
D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019873:Schnitzler Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology