title: Adult-onset Still's disease: Advances in the treatment.
abstract: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder mainly characterized by persistent high spiking fevers, evanescent rash, and joint involvement. The pathogenesis of AOSD is only partially known, but pro-inflammatory cytokines such as tumor necrosis factor (TNF)-α, interleukin (IL)-1, IL-6, IL-18, and IFN-γ seem to play a major role in this disorder. AOSD is at the crossroad of auto-inflammatory syndromes and autoimmune diseases. It is diagnosed by exclusion to determine the presence of high serum ferritin levels, which is usually >1000 μg/L. AOSD is generally treated with non-steroidal anti-inflammatory drugs, corticosteroids, and disease-modifying anti-rheumatic drugs (DMARDs). Although information on biologic therapy in the management of AOSD is scarce, these drugs represent a major breakthrough in the management of patients with AOSD refractory to corticosteroids or conventional DMARDs or in patients presenting life-threatening manifestations. In this regard, TNF-α, IL-1, and IL-6 antagonists had been proved effective in patients with AOSD.
authors.affiliation: Rheumatology Division, Hospital de La Princesa, IIS-IPrincesa, c/ Diego de León 62, 28006 Madrid, Spain; Universidad Autónoma de Madrid (UAM), Madrid, Spain. Electronic address: scastas@gmail.com.
authors.forename: Santos
authors.initials: S
authors.lastname: Castañeda
authors.affiliation: Rheumatology Division, Hospital Universitario Marqués de Valdecilla, Avda. de Valdecilla, s/n, 39008 Santander, Cantabria, Spain. Electronic address: rblanco@humv.es.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Blanco
authors.affiliation: Rheumatology Division, Hospital Universitario Marqués de Valdecilla, Avda. de Valdecilla, s/n, 39008 Santander, Cantabria, Spain; Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, University of Cantabria, IDIVAL, Avda. de Valdecilla, s/n, 39008 Santander, Spain. Electronic address: miguelaggay@hotmail.com.
authors.forename: Miguel A
authors.initials: MA
authors.lastname: González-Gay
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D018501:Antirheumatic Agents / Q000627:therapeutic use; D006801:Humans; D016706:Still's Disease, Adult-Onset / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology
chemical_list: D000305:Adrenal Cortex Hormones; D000894:Anti-Inflammatory Agents, Non-Steroidal; D018501:Antirheumatic Agents
keywords: Adult-onset Still's disease; Anti-IL-6 receptor antibody-tocilizumab; Biologic agents; DMARDs; IL-1 receptor antagonist-anakinra; Macrophage activation syndrome; TNF-α inhibitors