title: Dramatic beneficial effect of interleukin-1 inhibitor treatment in patients with familial Mediterranean fever complicated with amyloidosis and renal failure.
abstract: BACKGROUND
  Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder, for which systemic AA amyloidosis is the major complication revealed most of the time by renal abnormalities. Current treatment is daily colchicine that prevents both recurrent inflammatory attacks and amyloidosis deposition in most patients. However, some patients still develop amyloidosis and renal failure. Functional studies suggest that interleukin (IL)-1 is implicated in the inflammatory reaction in FMF and therefore, IL-1 inhibitors could be a new approach to treat FMF. The aim of this series study was to evaluate anakinra in patients with FMF complicated with amyloidosis and renal failure.
  
  
  METHODS
  We studied a series of adult patients with FMF complicated with amyloidosis and treated with anakinra in one reference centre were reviewed. A search for published patients with FMF associated amyloidosis treated with anakinra was performed by screening PubMed.
  
  
  RESULTS
  We report four cases of patients with FMF-associated amyloidosis treated with anakinra and discuss the clinical pertinence of its use in these particular clinical settings.
  
  
  CONCLUSIONS
  Anakinra has a strong effect on both inflammatory attacks and general status in patients with FMF-associated amyloidosis. It may contribute to changing the prognosis of these patients. Long-term studies are needed to appreciate the effect of anakinra or other IL-1 inhibitors on the natural history of amyloidosis in these patients.
authors.affiliation: Service de Médecine Interne, Hôpital Tenon, Assistance Publique Hôpitaux de Paris (AP-HP), Université Paris 6 Pierre et Marie Curie (UPMC), Paris, France. katia.stankovic@tnn.aphp.fr
authors.forename: Katia
authors.initials: K
authors.lastname: Stankovic Stojanovic
authors.forename: Yahsou
authors.initials: Y
authors.lastname: Delmas
authors.forename: Pablo Ureña
authors.initials: PU
authors.lastname: Torres
authors.forename: Julie
authors.initials: J
authors.lastname: Peltier
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Pelle
authors.forename: Isabelle
authors.initials: I
authors.lastname: Jéru
authors.forename: Magali
authors.initials: M
authors.lastname: Colombat
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000209:etiology*; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D007375:Interleukin-1 / Q000037:antagonists & inhibitors*; D007676:Kidney Failure, Chronic / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016896:Treatment Outcome
chemical_list: D053590:Interleukin 1 Receptor Antagonist Protein; D007375:Interleukin-1