title: Biological agents in familial Mediterranean fever focusing on colchicine resistance and amyloidosis.
abstract: Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory syndrome. FMF is caused by mutations in the MEFV gene which encodes the pyrin protein. FMF is characterized by sporadic, paroxysmal attacks of fever and serosal inflammation, lasting 1-3 days. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis.5% to 10% of the patients with FMF are resistant or intolerant to colchicine. Colchicine resistant patients may receive biological therapies. Anti-interleukin-1 drugs are the most important agents of biological treatments. In this review, colchicine resistance and treatment options will be evaluated.
authors.forename: Betul
authors.initials: B
authors.lastname: Sozeri
authors.affiliation: Department of Pediatrics, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey. ozgurkc@istanbul.edu.tr.
authors.forename: Ozgur
authors.initials: O
authors.lastname: Kasapcopur
mesh_terms: D000686:Amyloidosis / Q000188:drug therapy*; D001685:Biological Factors / Q000494:pharmacology / Q000627:therapeutic use*; D003078:Colchicine / Q000494:pharmacology / Q000627:therapeutic use*; D004351:Drug Resistance / Q000187:drug effects; D010505:Familial Mediterranean Fever / Q000188:drug therapy*; D006801:Humans; D007375:Interleukin-1 / Q000378:metabolism
chemical_list: D001685:Biological Factors; D007375:Interleukin-1; D003078:Colchicine