title: Familial Mediterranean fever in Germany: clinical presentation and amyloidosis risk.
abstract: OBJECTIVE
  To characterize patients with familial Mediterranean fever (FMF) with and without AA amyloidosis living in Germany.
  
  
  METHODS
  Clinical and genetic data from 64 FMF patients were analysed for amyloidosis risk factors.
  
  
  RESULTS
  Fifty-five patients (85%) were of Turkish or Armenian origin. Thirty-one patients (48%) developed FMF symptoms before the age of 16 years. Sixteen patients (26%) became symptomatic after age 20. Symptoms reported were peritonitis (95%), fever (78%), pleuritis (59%), arthralgia (60%), arthritis (32%), erysipelas-like erythema (23%), and vasculitis (8%). FMF diagnosis was delayed for a median of 8.0 years. Genetic analysis confirmed M694V as the most prevalent Mediterranean fever (MEFV) gene mutation in 46 out of 59 patients (78%). M694V homozygosity was associated with an earlier FMF onset (median age 5.5 years, p = 0.0001) and a higher prevalence of peritonitis (p = 0.007) and pleuritis (p = 0.0007) compared to patients without an M694V mutation. AA amyloidosis was detected in 16 patients (25%) at a median age of 36.5 years and tended to be associated with a higher age at disease onset (p = 0.062) and a higher FMF activity score (p = 0.093). AA amyloidosis was significantly associated with a higher age at FMF diagnosis (p = 0.0022).
  
  
  CONCLUSIONS
  Clinical symptoms of FMF-affected migrants living in Germany resemble those observed in their home country. In particular, patients with an onset of FMF symptoms after age 20 and a later FMF diagnosis have a high risk of AA amyloidosis. Symptomatic patients who originate from countries with a higher FMF prevalence should be screened for FMF and proteinuria.
authors.affiliation: Division of Rheumatology, Department of Medicine V, University of Heidelberg, Heidelberg, Germany.
authors.forename: D
authors.initials: D
authors.lastname: Ebrahimi-Fakhari
authors.forename: S O
authors.initials: SO
authors.lastname: Schönland
authors.forename: U
authors.initials: U
authors.lastname: Hegenbart
authors.forename: P
authors.initials: P
authors.lastname: Lohse
authors.forename: J
authors.initials: J
authors.lastname: Beimler
authors.forename: L
authors.initials: L
authors.lastname: Wahlster
authors.forename: A D
authors.initials: AD
authors.lastname: Ho
authors.forename: H-M
authors.initials: HM
authors.lastname: Lorenz
authors.forename: N
authors.initials: N
authors.lastname: Blank
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis / Q000208:ethnology* / Q000235:genetics*; D003598:Cytoskeletal Proteins / Q000235:genetics*; D010505:Familial Mediterranean Fever / Q000175:diagnosis / Q000208:ethnology* / Q000235:genetics*; D005858:Germany / Q000453:epidemiology; D006801:Humans; D008875:Middle Aged; D010538:Peritonitis / Q000175:diagnosis / Q000208:ethnology / Q000235:genetics; D017354:Point Mutation / Q000235:genetics; D015995:Prevalence; D000071198:Pyrin; D012189:Retrospective Studies; D012307:Risk Factors; D014171:Transients and Migrants / Q000706:statistics & numerical data*; D055815:Young Adult
chemical_list: D003598:Cytoskeletal Proteins; C000604192:MEFV protein, human; D000071198:Pyrin