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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.

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  • Publisher Full Text
  • Authors

    Akar S, Yuksel F, Tunca M, Soysal O, Solmaz D, Gerdan V, Celik A, Sen G, Onen F, Akkoc N

    Source

    Medicine 91:3 2012 May pg 131-6

    MeSH

    Amyloidosis
    Cause of Death
    Colchicine
    Familial Mediterranean Fever
    Female
    Follow-Up Studies
    Humans
    Kaplan-Meier Estimate
    Male
    Prognosis
    Proportional Hazards Models
    Questionnaires
    Risk Factors
    Survival Analysis
    Turkey

    Pub Type(s)

    Journal Article

    Language

    eng

    PubMed ID

    22543627