Although progressive sensorineural hearing loss and the tendency to develop renal amyloidosis, with the increase of serum levels of amyloid A protein (SAA), are considered the most specific findings in MWS, atypical symptoms such as severe abdominal pain, recurrent genital and oral ulcers, and livedo reticularis have also been reported in some families (67, 68), together with the widely reported MWS/FCAS (69–73) and MWS/CINCA/NOMID (74–76) overlap features. Here, SAA1 is linked to CINCA syndrome.