title: Anakinra is safe and effective in controlling hyperimmunoglobulinaemia D syndrome-associated febrile crisis.
abstract: Hyper-IgD and periodic fever syndrome (HIDS) is a hereditary autoinflammatory syndrome, characterized by recurrent inflammatory attacks. Treatment of HIDS is difficult. Recently, the IL-1ra analogue anakinra was reported to be successful in aborting the IgD inflammatory attacks in a vaccination model. We report a clinical case of spectacular reduction of febrile attacks in a severe HIDS patient.
authors.affiliation: AP-HM Timone-Enfants, F-13385, Marseilles Cedex 05, France.
authors.forename: M
authors.initials: M
authors.lastname: Cailliez
authors.forename: F
authors.initials: F
authors.lastname: Garaix
authors.forename: C
authors.initials: C
authors.lastname: Rousset-Rouvière
authors.forename: D
authors.initials: D
authors.lastname: Bruno
authors.forename: I
authors.initials: I
authors.lastname: Kone-Paut
authors.forename: J
authors.initials: J
authors.lastname: Sarles
authors.forename: B
authors.initials: B
authors.lastname: Chabrol
authors.forename: M
authors.initials: M
authors.lastname: Tsimaratos
mesh_terms: D005334:Fever / Q000209:etiology / Q000628:therapy*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications* / Q000628:therapy*; D007072:Immunoglobulin D / Q000737:chemistry; D007249:Inflammation; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D013577:Syndrome
chemical_list: D007072:Immunoglobulin D; D053590:Interleukin 1 Receptor Antagonist Protein