title: Autoinflammatory syndromes.
abstract: Autoinflammatory syndromes comprise a diagnostically challenging group of systemic inflammatory disorders uniquely related by (1) dysregulation of innate immunity, (2) inflammasome activation, (3) dramatic clinical features (high fevers, neutrophilic rashes, and bone or synovial involvement), (4) impressive acute phase responses, and (5) effective treatment with cytokine inhibitors. This review details some of the more common autoinflammatory disorders, their distinguishing features and dermatologic manifestations, and how an accurate diagnosis can be established in patients presenting with periodic or intermittent febrile disorders.
authors.affiliation: Baylor Research Institute, Rheumatology Research, 9900 North Central Expressway, Suite 550, Dallas, TX 75231, USA. cushj@msn.com
authors.forename: John J
authors.initials: JJ
authors.lastname: Cush
mesh_terms: D001171:Arthritis, Juvenile / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D016207:Cytokines / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D005334:Fever / Q000188:drug therapy / Q000276:immunology; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007113:Immunity, Innate / Q000276:immunology; D007249:Inflammation / Q000188:drug therapy / Q000276:immunology; D016706:Still's Disease, Adult-Onset / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D013577:Syndrome
chemical_list: D016207:Cytokines
keywords: Autoinflammatory; CAPS; Periodic fever; Still disease