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10.1186/ar4237

http://scihub22266oqcxt.onion/10.1186/ar4237
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C4060562!4060562!23718630
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suck abstract from ncbi


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pmid23718630      Arthritis+Res+Ther 2013 ; 15 (3): R64
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  • Treatment of Muckle-Wells syndrome: analysis of two IL-1-blocking regimens #MMPMID23718630
  • Kuemmerle-Deschner JB; Wittkowski H; Tyrrell PN; Koetter I; Lohse P; Ummenhofer K; Reess F; Hansmann S; Koitschev A; Deuter C; Bialkowski A; Foell D; Benseler SM
  • Arthritis Res Ther 2013[]; 15 (3): R64 PMID23718630show ga
  • Objectives: Muckle-Wells syndrome (MWS) is an autoinflammatory disease characterized by excessive interleukin-1 (IL-1) release, resulting in recurrent fevers, sensorineural hearing loss, and amyloidosis. IL-1 inhibition with anakinra, an IL-1 receptor antagonist, improves clinical symptoms and inflammatory markers. Subclinical disease activity is commonly observed. Canakinumab, a fully human IgG1 anti-IL-1? monoclonal antibody, can abolish excess IL-1?. The study aim was to analyze the efficacy and safety of these two anti-IL-1 therapies. Methods: Two cohorts of patients with severe MWS and confirmed NLRP3 mutation were treated with anakinra and/or canakinumab. Clinical and laboratory features including ESR, CRP, SAA, and the neutrophil marker S100A12 were determined serially. Disease activity was captured by MWS disease activity scores (MWS-DAS). Remission was defined as MWS-DAS ?5 plus normal CRP and SAA. Treatment efficacy and safety were analyzed. Results: The study included 12 anakinra- and 14 canakinumab-treated patients; the median age was 33.5 years (3.0 years to 72.0 years); 57% were female patients. Both treatment regimens led to a significant reduction of clinical disease activity and inflammatory markers. At last follow-up, 75% of anakinra-treated and 93% of canakinumab-treated patients achieved remission. During follow-up, S100A12 levels mirrored recurrence of disease activity. Both treatment regimens had favorable safety profiles. Conclusions: IL-1 blockade is an effective and safe treatment in MWS patients. MWS-DAS in combination with MWS inflammatory markers provides an excellent monitoring tool set. Canakinumab led to a sustained control of disease activity even after secondary failure of anakinra therapy. S100A12 may be a sensitive marker to detect subclinical disease activity.
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