URTICARIA, ARTHRALGIA, AND NEPHROPATHY WITHOUT AMYLOIDOSIS - ANOTHER VARIANT OF THE MUCKLE-WELLS SYNDROME

The term Muckle-Wells syndrome (MWS) describes an autosomal dominant d isorder characterised by various combinations of urticaria, sensorineu ral deafness, amyloidosis, arthralgia and skeletal abnormalities. We d escribe a family with nephropathy and several symptoms of MWS, but no evidence of deafness or amyloidosis. Since nephropathy without amyloid osis has never been reported in MWS, but deafness is a feature of all reported pedigrees, we conclude that members of this family have a pre viously unreported inherited predisposition to urticaria, arthralgia a nd nephropathy which is distinct from the MWS phenotype.