title: [Pathophysiology, subtypes, and treatments of adult-onset Still's disease: An update].
abstract: Adult-onset Still's disease is a rare and difficult to diagnose multisystemic disorder considered as a multigenic autoinflammatory syndrome. Its immunopathogenesis seems to be at the crossroads between inflammasomopathies and hemophagocytic lymphohistiocytosis, the most severe manifestation of the disease. According to recent insights in the pathophysiology and thanks to cohort studies and therapeutic trials, two phenotypes of adult-onset Still's disease may be distinguished: a systemic pattern, initially highly symptomatic and with a higher risk to exhibit life-threatening complications such as reactive hemophagocytic lymphohistiocytosis, where interleukin-1 blockade seems to be very effective, a chronic articular pattern, more indolent with arthritis in the foreground and less severe systemic manifestations, which would threat functional outcome and where interleukin-6 blockade seems to be more effective. This review focuses on these data.
authors.affiliation: Service de médecine interne, hôpital de la Croix-Rousse, hospices civils de Lyon, 69004 Lyon, France; Université Lyon I, 69100 Villeurbanne, France; Université de Lyon, 69000 Lyon, France.
authors.forename: M
authors.initials: M
authors.lastname: Gerfaud-Valentin
authors.affiliation: Service de médecine interne, hôpital de la Croix-Rousse, hospices civils de Lyon, 69004 Lyon, France; Université Lyon I, 69100 Villeurbanne, France; Université de Lyon, 69000 Lyon, France. Electronic address: pascal.seve@chu-lyon.fr.
authors.forename: P
authors.initials: P
authors.lastname: Sève
authors.affiliation: Université Lyon I, 69100 Villeurbanne, France; Université de Lyon, 69000 Lyon, France; Service de médecine interne, hôpital Édouard-Herriot, hospices civils de Lyon, 69003 Lyon, France.
authors.forename: A
authors.initials: A
authors.lastname: Hot
authors.affiliation: Service de médecine interne, hôpital de la Croix-Rousse, hospices civils de Lyon, 69004 Lyon, France; Université Lyon I, 69100 Villeurbanne, France; Université de Lyon, 69000 Lyon, France.
authors.forename: C
authors.initials: C
authors.lastname: Broussolle
authors.affiliation: Service de médecine interne, hôpital de la Croix-Rousse, hospices civils de Lyon, 69004 Lyon, France; Inserm U1111, Centre international de recherche en infectiologie, 69365 Lyon, France; Département de biochimie, université de Lausanne, 1006 Epalinges, Suisse.
authors.forename: Y
authors.initials: Y
authors.lastname: Jamilloux
mesh_terms: D000328:Adult; D018450:Disease Progression; D006801:Humans; D012307:Risk Factors; D016706:Still's Disease, Adult-Onset / Q000145:classification* / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*
keywords: Adult-onset Still's disease; Autoinflammatory syndrome; Interleukin-1; Interleukin-6; Interleukine-1; Interleukine-6; Maladie de Still de l’adulte; Syndrome auto-inflammatoire