Sammanfattning
Schnitzler syndrome is characterised by monoclonal IgM gammopathy, urticaria, recurrent fever, evidence of inflammation, bone pain, arthralgia and occasionally appears in combination with lymphadenopathy and/or hepatosplenomegaly. This is the first case of Schnitzler syndrome to be published in Sweden. However uncommon the case is, its importance lies in helping to diagnose yet undiagnosed cases with occasionally disabling symptoms. While effective treatment with minor side effects is achieved with anakinra, the optimal dosage for a long-term treatment and the potential risks involved with a lifelong treatment are still unclear.