Unbound MEDLINE

Risk factors for amyloidosis and impact of kidney transplantation on the course of familial Mediterranean fever.

Abstract

BACKGROUND
Amyloidosis of familial Mediterranean fever (FMF) may lead to end-stage renal failure, culminating in kidney transplantation in some patients.
OBJECTIVES
To assess demographic, clinical and genetic risk factors for the development of FMF amyloidosis in a subset of kidney-transplanted patients and to evaluate the impact of transplantation on the FMF course.
METHODS
Demographic, clinical and genetic data were abstracted from the files, interviews and examinations of 16 kidney-transplanted FMF amyloidosis patients and compared with the data of 18 FMF patients without amyloidosis.
RESULTS
Age at disease onset and clinical severity of the FMF amyloidosis patients prior to transplantation were similar to FMF patients without amyloidosis. Compliance with colchicine treatment, however, was much lower (50% vs. 98%). Posttransplantation, FMF amyloidosis patients experienced fewer of the typical serosal attacks than did their counterparts (mean 2214 days since last attack vs. 143 days). Patients with FMF amyloidosis carried only M694V mutations in the FMF gene, while FMF without amyloidosis featured other mutations as well.
CONCLUSIONS
Compliance with treatment and genetic makeup but not severity of FMF constitutes major risk factors for the development of amyloidosis in FMF. Transplantation seems to prevent FMF attacks. The protective role of immunosuppressive therapy cannot be excluded.

Links

  • Publisher Full Text

    • Authors

    Ben-Zvi I, Danilesko I, Yahalom G, Kukuy O, Rahamimov R, Livneh A, Kivity S

    Institution

    Rheumatology Unit, Zabludowicz Center of Autoimmune Disease, Tel Hashomer, Israel.

    Source

    The Israel Medical Association journal : IMAJ 14:4 2012 Apr pg 221-4

    MeSH

    Adult
    Amyloidosis
    Case-Control Studies
    Colchicine
    Cross-Sectional Studies
    Cytoskeletal Proteins
    Ethnic Groups
    Familial Mediterranean Fever
    Female
    Homozygote
    Humans
    Immunosuppressive Agents
    Kidney Diseases
    Kidney Transplantation
    Male
    Medication Adherence
    Middle Aged
    Mutation
    Retrospective Studies
    Risk Factors
    Sex Factors

    Pub Type(s)

    Journal Article

    Language

    eng

    PubMed ID

    22675837