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10.2147/OARRR.S114447

http://scihub22266oqcxt.onion/10.2147/OARRR.S114447
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C5513904!5513904 !28744167
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suck abstract from ncbi


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pmid28744167
      Open+Access+Rheumatol 2017 ; 9 (ä): 123-129
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  • Muckle-Wells syndrome: clinical perspectives #MMPMID28744167
  • Tran TA
  • Open Access Rheumatol 2017[]; 9 (ä): 123-129 PMID28744167 show ga
  • Muckle-Wells syndrome (MWS) is a rare autoinflammatory disorder. It is due to NLRP3 gene mutations, responsible for excessive caspase-1 activation and interleukin 1? processing. MWS is the intermediate phenotype of severity of cryopyrin-associated periodic syndrome. Urticarial rash, conjunctivitis, recurrent fever, arthralgia, and fatigue are the main clinical manifestations of MWS. Yet, sensorineural hearing loss and renal amyloidosis can occur after long term evolution. Patients' quality of life has been drastically improved with the advent of IL-1 inhibitors. This review reports recent findings in MWS, particularly genotype/phenotype correlation, and discusses the clinical perspectives of this disease in a time of efficient treatment.
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