Article

Schnitzler's syndrome with neurological findings

Technische Universität Dresden, Dresden, Saxony, Germany
Journal of Neurology (Impact Factor: 3.84). 11/2003; 250(10):1248-50. DOI: 10.1007/s00415-003-0169-2
Source: PubMed
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    ABSTRACT: Schnitzler syndrome (SS) is a rare clinical entity, which belongs to the spectrum of monoclonal gammapathy-associated systemic disorders. Its pathophysiology remains elusive, even if it is tempting to consider it as a late onset and probably acquired auto-inflammatory syndrome. SS mainly occurs in the fifth and sixth decade, and present with an urticariform rash with periodic fever and/or osteoarticular pain. Systemic inflammation and monoclonal gammapathy (overwhelmingly IgM kappa) are constant features. SS is a chronic disease, which can severely impair quality of life of the affected individuals. Many drugs have been used and proved disappointing. In the last few years, accumulating reports provided evidence for the dramatic efficacy of anakinra, which has revolutionized the management of most severe cases. The main long-term threat to these patients is to develop a lymphoproliferative disorder (mainly Waldenström's macroglobulinemia). The mechanisms underlying the different facets of the disease remain to be elucidated.
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    ABSTRACT: Schnitzler's syndrome is an autoinflammatory disorder characterized by the association of a monoclonal IgM (or IgG) gammopathy, a chronic urticarial rash, and signs and symptoms of systemic inflammation, including fever, arthralgias and bone pain. It was first described in 1972. This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have been reported to date. Also, the results of skin histology, bone imaging, laboratory investigations, and studies of the pathogenesis will be discussed, including the pivotal role of interleukin-1 beta in this disorder.
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    ABSTRACT: Le syndrome de Schnitzler associe une gammapathie monoclonale de type IgM avec urticaire, fièvre intermittente, syndrome inflammatoire, douleurs osseuses, arthralgies et parfois, des adénopathies et/ou une hépatosplénomégalie. Il est rare, puisque seul 80 cas environ ont été rapportés. Le pronostic est dominé par le risque de survenue d’une hémopathie maligne. L’efficacité spectaculaire des inhibiteurs de l’IL1 dans des observations récentes offre de nouvelles perspectives pour la compréhension de la physiopathologie.
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