title: Adult-onset Still's disease and chronic recurrent multifocal osteomyelitis: a hitherto undescribed manifestation of autoinflammation.
abstract: Still's disease and chronic recurrent multifocal osteomyelitis (CRMO) are febrile rheumatic diseases of unknown etiology, which predominantly affect children but can also have their initial manifestation in adults. Both can present as intermittent, relapsing episodes and are considered potential candidates within the expanding spectrum of autoinflammatory disorders, although no genetic abnormalities have been described for either of them. Here, we describe a man with an initial manifestation of abacterial multifocal osteitis at the age of 41. During a relapsing-remitting course of his illness, he increasingly developed symptoms of adult-onset Still's disease (AOSD), and the diagnosis was established according to the Yamaguchi criteria. When treated with anakinra, not only the acute symptoms disappeared promptly, but also the osteitis went into complete remission. This is to our knowledge the first description of a simultaneous occurrence of these two manifestations of autoinflammation in adulthood.
authors.affiliation: Department of Medicine 3, University Erlangen-Nuremberg, Krankenhausstr. 12, 91054, Erlangen, Germany. juergen.rech@uk-erlangen.de
authors.forename: J
authors.initials: J
authors.lastname: Rech
authors.forename: B
authors.initials: B
authors.lastname: Manger
authors.forename: B
authors.initials: B
authors.lastname: Lang
authors.forename: G
authors.initials: G
authors.lastname: Schett
authors.forename: M
authors.initials: M
authors.lastname: Wilhelm
authors.forename: J
authors.initials: J
authors.lastname: Birkmann
mesh_terms: D000328:Adult; D015551:Autoimmunity; D001706:Biopsy; D001853:Bone Marrow / Q000276:immunology / Q000473:pathology; D001856:Bone Marrow Examination; D001842:Bone and Bones / Q000000981:diagnostic imaging / Q000276:immunology; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D008297:Male; D010019:Osteomyelitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D011877:Radionuclide Imaging; D012074:Remission Induction; D016706:Still's Disease, Adult-Onset / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D016896:Treatment Outcome
chemical_list: D007155:Immunologic Factors; D053590:Interleukin 1 Receptor Antagonist Protein