Schnitzler’s syndrome: lessons from 281 cases
نویسنده
چکیده
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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Schnitzler’s syndrome: lessons from 281 cases
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 be...
متن کاملSchnitzler's syndrome without monoclonal gammopathy.
Sir, Schnitzler’s syndrome was first reported in 1972 and comprises chronic non-pruritic urticaria associated with intermittent fever, arthralgia, bone pain, hyperostosis, increased erythrocyte sedimentation rate (ESR) and a monoclonal IgM gammopathy (1). It is a rare and probably underdiagnosed disorder: so far about 50 cases have been reported in the literature. The aetiology of the syndrome ...
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Methods In an open-label, single-treatment arm trial, eight patients with Schnitzler’s syndrome received monthly injections with 150 mg canakinumab subcutaneously for 6 months, followed by a 3-month observation period. Primary outcome was complete or clinical remission at day 14. Secondary outcome measures included inflammatory markers, quality of life, time to relapse, safety and tolerability.
متن کاملSchnitzler’s Syndrome
Schnitzler’s syndrome is a rare clinical condition characterized by chronic urticaria, intermittent fever, bone pain, arthralgia or arthritis, and monoclonal immunoglobulin M (IgM) gammopathy. Here we describe a 64-year-old Taiwanese male with a long history of arthralgia, fever, and chronic urticaria with severe pruritus. The IgMmonoclonal component in the serum was detected 2 years after the ...
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Introduction Schnitzler’s syndrome (SchS) is an autoinflammatory disorder, characterized by chronic urticaria, fever, gammopathy and bone pain. The etiology is unknown, but interleukin-1 (IL-1) inhibition is very effective, like in the cryopyrin associated periodic syndrome (CAPS), that is caused by activating NLRP3 mutations. Previously, a V198M mutation in NLRP3 was reported in one patient wi...
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