Autosomal dominant familial Mediterranean feverlike syndrome

We report a syndrome characterized by recurrent episodes of fever and serositis in an Austrian family. Three family members over two successive generations were af- fected. The febrile episodes had their onset at the age of 11-12 years, lasted 1-5 weeks, and occurred in in- tervals of 6-24 months. While the disorder resembles familial Mediter- ranean fever (FMF) clinically, ethnic distribution and other features sug- gest a distinct entity. Clinically, the attacks last longer than the usual FMF attacks, and in the male pa- tients are associated with scrotal in- flammation. Genetically, the disorder appears to be inherited as an autoso- mal dominant syndrome, whereas FMF is autosomal recessive. Molec- ular analysis made the involvement of a gene in the FMF region of chro- mosome 16p 13.3 highly unlikely. ConcluSiOn AnAust~an family with recurren~ fever Sy~dr0me iS re' portedl Ethn~ityl blini~al fea~reS; and mot;cuiar studies point tO: a distinc{ dliNCal enfityl