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Schnitzlers syndrom er en diagnostisk udfordring

Pernille Hurup Duhn1, Simon Francis Thomsen2 & Henrik Nordin1 1) Klinik for Infektionsmedicin og Reumatologi, Rigshospitalet 2) Dermatologisk Afdeling, Bispebjerg Hospital Ugeskr Læger 2015;177:V12140756
Ugeskr Læger 2015;177:V12140756
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Schnitzler syndrome is a diagnostic challenge
Schnitzler syndrome (SS) is a rare autoinflammatory disorder characterized by a chronic urticarial rash and a monoclonal immunoglobulin M gammopathy, accompanied by recurrent fever, lymphadenopathy, arthralgia or arthritis, hepato- or splenomegaly and elevated levels of markers of systemic inflam­mation. Because patients often present to various spe­cialists with different symptoms the syndrome is often undiagnosed, and it can take years before the correct diagnosis is made. Treatment with interleukin-1 receptor antagonists has a rapid effect on SS.
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