Long-Term Complications of Familial Mediterranean Fever

Abstract

The recurrent infl ammatory attacks in FMF cause intense acute symptoms and confer a markedly increased relative risk of developing AA amyloidosis and eventually end-stage renal disease. Prior to the colchicine era, this was a major scourge particularly around the Eastern Mediterranean. Other potential complications of FMF include destructive arthritis, adhesions, subfertility, and vasculitis. Fortunately, these remain very rare, and with long-term treatment, recent studies suggest that both complications and mortality for FMF appear very close to age-matched healthy controls. MortalityThe most comprehensive study of mortality in FMF came from Israel in 2013 and compared 1,225 individuals with FMF to 1,243,125 controls among military service recruits (aged 16-20) from 1993 to 1997 [ 1 ]. Over 30 years of follow-up, death rates were signifi cantly higher in individuals with FMF: 8.73 compared to 4.32 per 10,000 person-years and hazard ratio 1.705 for males and 2.48 for females. Renal AA amyloidosis seemed to be the single commonest contributor, reported in 35 and 60 % of deaths in men and women, respectively. There was no suggestion that malignancy rates differed between the FMF cases and controls. This study only included individuals who were healthy enough for military conscription as young adults, and consequently, their mortality rates were lower than that recently reported for the general Israeli population in the age range 25-49 years