The Schnitzler syndrome: chronic urticaria and monoclonal gammopathy--an autoinflammatory syndrome?
Schnitzler syndrome describes the simultaneous occurrence of monoclonal gammopathy and chronic urticaria with at least two additional minor symptoms (arthralgia, bone pain, fever of uncertain origin, hepato- or splenomegaly, lymphadenopathy, increased erythrocyte sedimentation rate, leukocytosis/thrombocytosis or increased bone density). Schnitzler syndrome is not wellknown and very likely under-recognized. Comprehensive diagnostic examinations are necessary to rule out other diseases, especially those of hematologic origin. Close interdisciplinary collaboration is mandatory. The etiology of Schnitzler syndrome is unclear, but the rapid response to the interleukin-1 receptor inhibitor anakinra underlines the pivotal role which the proinflammatory cytokine interleukin-1 may play in the pathophysiology of this potentially autoinflammatory disorder.
Eiling
Elisabeth
E
Department of Dermatology, Venereology and Allergy, University Hospital Schleswig-Holstein, Campus Kiel, Germany.
Schröder
Johann O
JO
Gross
Wolfgang L
WL
Kreiselmaier
Inga
I
Mrowietz
Ulrich
U
Schwarz
Thomas
T
D001327:Autoimmune Diseases / Q000145:classification / Q000175:diagnosis* / Q000628:therapy; D003872:Dermatitis / Q000145:classification / Q000175:diagnosis* / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000145:classification / Q000175:diagnosis* / Q000628:therapy; D019873:Schnitzler Syndrome / Q000145:classification / Q000175:diagnosis* / Q000628:therapy; D014581:Urticaria / Q000145:classification / Q000175:diagnosis* / Q000628:therapy