title: Mortality risk factors associated with familial Mediterranean fever among a cohort of 1.25 million adolescents.
abstract: OBJECTIVE
  There are limited data on long-term comorbidities and mortality among patients with familial Mediterranean fever (FMF). Our objective was to evaluate comorbidities and death rates among individuals with FMF.
  
  
  METHODS
  We studied a nationwide, population-based, retrospective cohort of 1225 individuals with FMF (59% men) in a database of 1 244 350 adolescents (16-20 years of age) medically evaluated for military service between 1973 and 1997. This cohort was linked with the national mortality, cancer and end-stage renal disease (ESRD) registries in Israel. Study outcomes were all-cause mortality, occurrence of ESRD and malignancy by the age of 50 years.
  
  
  RESULTS
  During 30 years of follow-up, death rates were 8.73/10(4) versus 4.32/10(4) person-years in the FMF and control groups, respectively (p=0.002). In a multivariable analysis adjusted for age, birth year, socio-economic status, education, ethnicity and body mass index, FMF was associated with increased mortality in men (HR=1.705 (95% CI 1.059 to 2.745), p=0.028) and women (HR=2.48 (1.032 to 5.992), p=0.042). Renal amyloidosis accounted for 35% and 60% of deaths in men and women, respectively. FMF was not associated with an increased incidence of cancer.
  
  
  CONCLUSIONS
  FMF is associated with increased all-cause mortality that is likely attributed to reduced colchicine compliance or responsiveness. Individuals with FMF do not have an increased incidence of cancer. These results support the awareness among medical community to decrease the higher than average mortality rate among participants with FMF.
authors.affiliation: Department of Medicine, Chaim Sheba Medical Center, , Tel Hashomer, Israel.
authors.forename: Gilad
authors.initials: G
authors.lastname: Twig
authors.forename: Avi
authors.initials: A
authors.lastname: Livneh
authors.forename: Asaf
authors.initials: A
authors.lastname: Vivante
authors.forename: Arnon
authors.initials: A
authors.lastname: Afek
authors.forename: Ari
authors.initials: A
authors.lastname: Shamiss
authors.forename: Estela
authors.initials: E
authors.lastname: Derazne
authors.forename: Dorit
authors.initials: D
authors.lastname: Tzur
authors.forename: Ilan
authors.initials: I
authors.lastname: Ben-Zvi
authors.forename: Amir
authors.initials: A
authors.lastname: Tirosh
authors.forename: Micha
authors.initials: M
authors.lastname: Barchana
authors.forename: Tamy
authors.initials: T
authors.lastname: Shohat
authors.forename: Eliezer
authors.initials: E
authors.lastname: Golan
authors.forename: Howard
authors.initials: H
authors.lastname: Amital
mesh_terms: D000293:Adolescent; D000686:Amyloidosis / Q000209:etiology / Q000401:mortality; D015331:Cohort Studies; D015897:Comorbidity; D010505:Familial Mediterranean Fever / Q000150:complications / Q000401:mortality*; D005260:Female; D006801:Humans; D015994:Incidence; D007557:Israel / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D007674:Kidney Diseases / Q000209:etiology / Q000401:mortality; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000401:mortality; D008297:Male; D009369:Neoplasms / Q000453:epidemiology / Q000209:etiology; D012307:Risk Factors; D012923:Social Class; D055815:Young Adult
keywords: Amyloidosis; Epidemiology; Familial Mediterranean Fever