Document Detail


Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study.
MedLine Citation:
PMID:  15643295     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Familial Mediterranean fever (FMF) is an autosomal recessive disease that is prevalent among eastern Mediterranean populations, mainly non-Ashkenazi Jews, Armenians, Turks, and Arabs. Since a large proportion of all the FMF patients in the world live in Turkey, the Turkish FMF Study Group (FMF-TR) was founded to develop a patient registry database and analyze demographic, clinical, and genetic features. The cohort was composed of 2838 patients (mean age, 23.0 +/- 13.33 yr; range, 2-87 yr), with a male:female ratio of 1.2:1. There was a mean period of 6.9 +/- 7.65 years from disease onset to diagnosis; the period was about 2 years shorter for each decade since 1981. Ninety-four percent of patients were living in the central-western parts of the country; however, their familial origins (70% from the central-eastern and Black Sea regions) reflected not only the ongoing east to west migration, but also the historical roots of FMF in Turkey. Patients' clinical features included peritonitis (93.7%), fever (92.5%), arthritis (47.4%), pleuritis (31.2%), myalgia (39.6%), and erysipelas-like erythema (20.9%). Arthritis, arthralgia, myalgia, and erysipelas-like erythema were significantly more frequent (p < 0.001) among patients with disease onset before the age of 18 years. Genetic analysis of 1090 patients revealed that M694V was the most frequent mutation (51.4%), followed by M680I (14.4%) and V726A (8.6%). Patients with the M694V/M694V genotype were found to have an earlier age of onset and higher frequencies of arthritis and arthralgia compared with the other groups (both p < 0.001). In contrast to other reported studies, there was no correlation between amyloidosis and M694V homozygosity in this cohort. However, amyloidosis was still remarkably frequent in our patients (12.9%), and it was prevalent (27.8%) even among the 18 patients with a disease onset after age 40 years. Twenty-two patients (0.8%) had nonamyloid glomerular diseases. The high prevalence of vasculitides (0.9% for polyarteritis nodosa and 2.7% for Henoch-Schonlein purpura) and high frequency of pericarditis (1.4%) were striking findings in the cohort. Phenotype II cases (those patients with amyloidosis as the presenting or only manifestation of disease) were rare (0.3% or less). There was a high rate of a past diagnosis of acute rheumatic fever, which suggested a possible misdiagnosis in children with FMF presenting with recurrent arthritis. To our knowledge, this is the largest series of patients with FMF reported from 1 country. We describe the features of the disease in the Turkish population and show that amyloidosis is still a substantial problem.
Authors:
Mehmet Tunca; Servet Akar; Fatos Onen; Huri Ozdogan; Ozgur Kasapcopur; Fatos Yalcinkaya; Ercan Tutar; Seza Ozen; Rezan Topaloglu; Engin Yilmaz; Mustafa Arici; Aysin Bakkaloglu; Nesrin Besbas; Tekin Akpolat; Ayhan Dinc; Eren Erken;
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Publication Detail:
Type:  Journal Article; Multicenter Study    
Journal Detail:
Title:  Medicine     Volume:  84     ISSN:  0025-7974     ISO Abbreviation:  Medicine (Baltimore)     Publication Date:  2005 Jan 
Date Detail:
Created Date:  2005-01-11     Completed Date:  2005-03-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2985248R     Medline TA:  Medicine (Baltimore)     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1-11     Citation Subset:  AIM; IM    
Affiliation:
Dokuz Eylul University School of Medicine, Department of Internal Medicine, Izmir, Turkey. mehmet.tunca@deu.edu.tr
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Amyloidosis, Familial / etiology
Child
Child, Preschool
Colchicine / therapeutic use
Epidemiologic Methods
Familial Mediterranean Fever* / complications,  ethnology,  genetics
Female
Humans
Male
Middle Aged
Registries
Turkey / ethnology
Chemical
Reg. No./Substance:
64-86-8/Colchicine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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