title: Successful treatment of familial Mediterranean fever with Anakinra and outcome after renal transplantation.
abstract: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent episodes of fever and inflammation. The most severe complication of FMF is the development of AA amyloidosis, which can be life threatening. The only current effective treatment for FMF is colchicine. Regular prophylactic treatment with colchicine at a dose of 1-2 mg daily prevents or substantially reduces the clinical manifestations of FMF in at least 90% of cases. However, approximately 10% of patients are reported to be resistant or non-responsive to colchicine and in these cases there is no consensus as to which second line agents should be used. We describe the first case, to our knowledge, of a patient with FMF and end-stage renal failure due to AA amyloidosis, successfully treated with IL-1 receptor blockade. Our data suggest that the IL-1 receptor antagonist Anakinra (Kineret; r-metHuIL-1 ra) may represent a safe and effective therapy for the treatment of colchicine-resistant FMF, in patients requiring renal replacement therapy, with dialysis or transplantation.
authors.affiliation: 6th Medical Department with Nephrology and Dialysis, Wilhelminenspital, 1160 Vienna, Montleartstrasse 37, Austria. Christian.moser.md@aon.at
authors.forename: Christian
authors.initials: C
authors.lastname: Moser
authors.forename: Gudrun
authors.initials: G
authors.lastname: Pohl
authors.forename: Isabella
authors.initials: I
authors.lastname: Haslinger
authors.forename: Sylvia
authors.initials: S
authors.lastname: Knapp
authors.forename: Dorota
authors.initials: D
authors.lastname: Rowczenio
authors.forename: Tonia
authors.initials: T
authors.lastname: Russel
authors.forename: Helen J
authors.initials: HJ
authors.lastname: Lachmann
authors.forename: Ursula
authors.initials: U
authors.lastname: Lang
authors.forename: Josef
authors.initials: J
authors.lastname: Kovarik
mesh_terms: D000686:Amyloidosis / Q000209:etiology; D003078:Colchicine / Q000627:therapeutic use; D004351:Drug Resistance; D010505:Familial Mediterranean Fever / Q000150:complications / Q000188:drug therapy* / Q000601:surgery*; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
chemical_list: D053590:Interleukin 1 Receptor Antagonist Protein; D003078:Colchicine