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Canakinumab as rescue therapy in familial Mediterranean fever refractory to conventional treatment.

Abstract
Familial Mediterranean fever is an autosomal recessive autoinflammatory disorder mainly affecting Mediterranean populations, which is associated with mutations of the MEFV gene that encodes pyrin. Functional studies suggest that pyrin is implicated in the maturation and secretion of interleukin-1 (IL-1). The IL-1 receptor antagonist or anti-IL-1 monoclonal antibody may therefore represent a rational approach for the treatment of the rare patients who are refractory to conventional therapy. We report the case of a young female affected by familial Mediterranean fever who proved to be resistant to colchicine and was successfully treated with canakinumab.
AuthorsMirella Alpa, Dario Roccatello
JournalDrug design, development and therapy (Drug Des Devel Ther) Vol. 9 Pg. 1983-7 ( 2015) ISSN: 1177-8881 [Electronic] New Zealand
PMID25945034 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • Cytoskeletal Proteins
  • Interleukin 1 Receptor Antagonist Protein
  • Interleukin-1
  • MEFV protein, human
  • Pyrin
  • Receptors, Interleukin-1
  • canakinumab
  • Colchicine
Topics
  • Adult
  • Antibodies, Monoclonal (adverse effects, therapeutic use)
  • Antibodies, Monoclonal, Humanized
  • Colchicine (therapeutic use)
  • Cytoskeletal Proteins
  • Drug Resistance
  • Familial Mediterranean Fever (drug therapy)
  • Female
  • Humans
  • Interleukin 1 Receptor Antagonist Protein
  • Interleukin-1 (antagonists & inhibitors, metabolism)
  • Pyrin
  • Receptors, Interleukin-1 (antagonists & inhibitors)
  • Treatment Outcome

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