title: Familial Mediterranean fever: risk factors, causes of death, and prognosis in the colchicine era.
abstract: We assessed the risk factors and causes of death in patients with familial Mediterranean fever (FMF) in an era when colchicine is the standard therapy for all patients.This study included all FMF patients who had presented to any of the internal medicine, rheumatology, or nephrology clinics at Dokuz Eylul University Hospital between 1992 and 2009. Of the 650 patients with FMF identified, 587 (90.3%) had either a face-to-face (n = 380) or telephone (n = 193) interview, or were confirmed as deceased. A structured questionnaire was used to obtain socioeconomic and demographic data, presenting and cumulative clinical features, and disease severity scores.During the follow-up period mortality was analyzed by calculating age- and sex-standardized mortality ratio (SMR) according to the mortality statistics of the Turkish population. Factors predictive of mortality were evaluated using Kaplan-Meier and Cox proportional hazard models. Sixty-three (9.7%) patients whose initial demographic and major clinical characteristics were similar to the rest of the group could not be contacted during the study period.Most (94.2%) patients were on colchicine at the time of the study. Thirty-seven (6.3%) patients had biopsy-verified amyloidosis, and 44 (7.5%) had renal disease. During a median follow-up of 6 years, 14 patients (9 women) died, and amyloidosis and its related complications were the leading causes of death in 7 patients. Univariate analysis revealed that increasing age, coronary heart disease, hypertension, renal disease, and amyloidosis were associated with mortality. However, Cox regression analysis showed amyloidosis as the only significant predictor of mortality (p < 0.001). The overall patient survival rate was not significantly different from the age- and sex-matched Turkish general population (SMR, 1.48; 95% confidence interval, 0.817-2.49).Our findings suggest that although the survival of FMF patients in the colchicine era is comparable to that of the general population, renal involvement still predicts mortality.
authors.affiliation: From the Division of Rheumatology (SA, OS, DS, VG, GS, FO, NA) and Division of Nephrology (AC), Department of Internal Medicine (FY, MT), Dokuz Eylul University School of Medicine, Izmir, Turkey.
authors.forename: Servet
authors.initials: S
authors.lastname: Akar
authors.forename: Feride
authors.initials: F
authors.lastname: Yuksel
authors.forename: Mehmet
authors.initials: M
authors.lastname: Tunca
authors.forename: Ozgul
authors.initials: O
authors.lastname: Soysal
authors.forename: Dilek
authors.initials: D
authors.lastname: Solmaz
authors.forename: Vedat
authors.initials: V
authors.lastname: Gerdan
authors.forename: Ali
authors.initials: A
authors.lastname: Celik
authors.forename: Gercek
authors.initials: G
authors.lastname: Sen
authors.forename: Fatos
authors.initials: F
authors.lastname: Onen
authors.forename: Nurullah
authors.initials: N
authors.lastname: Akkoc
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000453:epidemiology*; D002423:Cause of Death; D003078:Colchicine / Q000627:therapeutic use*; D010505:Familial Mediterranean Fever / Q000150:complications / Q000188:drug therapy / Q000401:mortality*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D011379:Prognosis; D016016:Proportional Hazards Models; D012307:Risk Factors; D011795:Surveys and Questionnaires; D016019:Survival Analysis; D014421:Turkey
chemical_list: D003078:Colchicine