Schnitzler syndrome is a rare entity characterized by the simultaneous occurrence of monoclonal gammopathy and chronic urticaria with at least two additional minor symptoms (arthralgia, bone pain, recurrent fever, hepatosplenomegaly, lymphadenopathy, evidence of inflammation). The syndrome is not well-known and often underdiagnosed. The development of a haematological malignancy is the main complication. The aetiology is unclear, but the rapid response to the interleukin-1 receptor antagonist therapy underlines the pivotal role that IL-1 may play in the pathophysiology of the disease.
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