MEFV and primary systemic amyloidosis: In this regard, three different FMF types have been suggested: type 1 FMF refers to the presence of overt clinical inflammatory disease; type 2 FMF presents with systemic amyloidosis in otherwise asymptomatic subjects; and type 3 FMF is related to the absence of inflammatory manifestations and systemic amyloidosis in subjects carrying MEFV mutations [31].