Abstract
Background The Muckle-Wells syndrome (MWS) is a rare inherited disease and belongs to the group of cryopyrin-associated periodic syndromes (CAPS). Recurrent fever attacks, myalgia, arthralgia, urticarial rash, headache, conjunctivitis, sensorineural deafness and a severe fatigue syndrome are the typical symptoms of MWS. Due to an unregulated production of IL1 a continuous formation of serum amyloid leads ultimately to the development of AA-amyloidosis, which is life-threatening and in some cases the fatal complication of MWS.
Highlights
The Muckle-Wells syndrome (MWS) is a rare inherited disease and belongs to the group of cryopyrin-associated periodic syndromes (CAPS)
Myalgia, arthralgia, urticarial rash, headache, conjunctivitis, sensorineural deafness and a severe fatigue syndrome are the typical symptoms of MWS
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Summary
The Muckle-Wells syndrome (MWS) is a rare inherited disease and belongs to the group of cryopyrin-associated periodic syndromes (CAPS). Myalgia, arthralgia, urticarial rash, headache, conjunctivitis, sensorineural deafness and a severe fatigue syndrome are the typical symptoms of MWS. Due to an unregulated production of IL1 a continuous formation of serum amyloid leads to the development of AA-amyloidosis, which is life-threatening and in some cases the fatal complication of MWS
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