Familial Mediterranean fever in Germany

• Verfasst von: Ebrahimi-Fakhari, Darius [VerfasserIn]
• Verfasst von: Schönland, Stefan [VerfasserIn]
• Verfasst von: Hegenbart, Ute [VerfasserIn]
• Verfasst von: Beimler, Jörg [VerfasserIn]
• Verfasst von: Wahlster, Lara [VerfasserIn]
• Verfasst von: Ho, Anthony Dick [VerfasserIn]
• Verfasst von: Lorenz, Hanns-Martin [VerfasserIn]
• Verfasst von: Blank, Norbert [VerfasserIn]
• Titel: Familial Mediterranean fever in Germany
• Titelzusatz: clinical presentation and amyloidosis risk
• Verf.angabe: D. Ebrahimi-Fakhari, S.O. Schönland, U. Hegenbart, P. Lohse, J. Beimler, L. Wahlster, A.D. Ho, H.-M. Lorenz & N. Blank
• Jahr: 2013
• Jahr des Originals: 2012
• Umfang: 8 S.
• Fussnoten: Published online: 08 Nov 2012 ; Gesehen am 16.04.2019
• Titel Quelle: Enthalten in: Scandinavian journal of rheumatology
• Ort Quelle: Abingdon : Taylor & Francis, 1972
• Jahr Quelle: 2013
• Band/Heft Quelle: 42(2013), 1, Seite 52-58
• ISSN Quelle: 1502-7732
• DOI: doi:10.3109/03009742.2012.714796
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1663297215

Abstract

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.