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Familial Mediterranean fever in Germany: clinical presentation and amyloidosis risk

Objective: To characterize patients with familial Mediterranean fever (FMF) with and without AA amyloidosis living in Germany.Method: 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. Thirt...

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Hauptverfasser: Ebrahimi-Fakhari, Darius (VerfasserIn) , Schönland, Stefan (VerfasserIn) , Hegenbart, Ute (VerfasserIn) , Beimler, Jörg (VerfasserIn) , Wahlster, Lara (VerfasserIn) , Ho, Anthony Dick (VerfasserIn) , Lorenz, Hanns-Martin (VerfasserIn) , Blank, Norbert (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 2013
In: Scandinavian journal of rheumatology
Year: 2012, Jahrgang: 42, Heft: 1, Pages: 52-58
ISSN:1502-7732
DOI:10.3109/03009742.2012.714796
Online-Zugang:Verlag, Volltext: https://doi.org/10.3109/03009742.2012.714796
Volltext
Verfasserangaben:D. Ebrahimi-Fakhari, S.O. Schönland, U. Hegenbart, P. Lohse, J. Beimler, L. Wahlster, A.D. Ho, H.-M. Lorenz & N. Blank

MARC

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520 |a Objective: To characterize patients with familial Mediterranean fever (FMF) with and without AA amyloidosis living in Germany.Method: 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. 
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