title: Review of autoinflammatory diseases, with a special focus on periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome.
abstract: There have been remarkable developments in the field of autoinflammatory diseases over the last 20 years. Research has led to definitions of new conditions, increased understanding of disease mechanisms and specific treatment. The polygenic autoinflammatory condition of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is the most common autoinflammatory disorder among children in many parts of the world. The clinical features often include clockwork regularity of episodes, prompt responses to corticosteroids and therapeutic effects of tonsillectomy, but the disease mechanisms are largely unknown.
  
  
  CONCLUSIONS
  This review discusses the emerging understanding of autoinflammatory diseases, with special emphasis on PFAPA.
authors.affiliation: Department of Pediatrics, NU-Hospital Group, Uddevalla, Sweden. per.wekell@vgregion.se.|Department of Pediatrics, Institute of Clinical Sciences, University of Gothenburg, Gothenburg, Sweden. per.wekell@vgregion.se.
authors.forename: Per
authors.initials: P
authors.lastname: Wekell
authors.affiliation: Department of Rheumatology and Inflammation Research, Institute of Medicine, University of Gothenburg, Gothenburg, Sweden.
authors.forename: Anna
authors.initials: A
authors.lastname: Karlsson
authors.affiliation: Department of Pediatrics, Institute of Clinical Sciences, University of Gothenburg, Gothenburg, Sweden.|The Queen Silvia Children's Hospital, Gothenburg, Sweden.
authors.forename: Stefan
authors.initials: S
authors.lastname: Berg
authors.affiliation: Department of Pediatrics, Institute of Clinical Sciences, University of Gothenburg, Gothenburg, Sweden.|The Queen Silvia Children's Hospital, Gothenburg, Sweden.
authors.forename: Anders
authors.initials: A
authors.lastname: Fasth
mesh_terms: D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007154:Immune System Diseases; D007113:Immunity, Innate; D013577:Syndrome
keywords: Aphthous stomatitis; Autoinflammatory diseases; Disease mechanisms; Periodic fever; Pharyngitis and cervical adenitis