title: Successful treatment of macrophage activation syndrome complicating adult Still disease with anakinra.
abstract: A previously healthy 20-year-old man presented with adult Still disease (ASD). He developed life-threatening macrophage activation syndrome (MAS), which was refractory to standard immunosuppression but responded dramatically to the IL-1 receptor antagonist anakinra. Subsequent immunological investigations included assessment of the perforin expression of natural killer (NK) cells and CD8+ T cells, which confirmed MAS.
authors.affiliation: Department of Internal Medicine, Royal Perth Hospital, Perth, Western Australia, Australia. nelson.loh@health.wa.gov.au
authors.forename: N K
authors.initials: NK
authors.lastname: Loh
authors.forename: M
authors.initials: M
authors.lastname: Lucas
authors.forename: S
authors.initials: S
authors.lastname: Fernandez
authors.forename: D
authors.initials: D
authors.lastname: Prentice
mesh_terms: D018501:Antirheumatic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D005434:Flow Cytometry; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000008:administration & dosage / Q000627:therapeutic use*; D007694:Killer Cells, Natural; D018655:Lymphocyte Count; D055501:Macrophage Activation Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D008297:Male; D016706:Still's Disease, Adult-Onset / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D055815:Young Adult
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein