title: Lesson of the month 1: Autoinflammatory syndromes - an unusual cause of pyrexia of unknown origin.
abstract: Autoinflammatory diseases are disorders of innate immunity and are characterised by recurring and unprovoked episodes of inflammation. We present a case of episodic pyrexia, associated with a significant inflammatory response, in a young man in whom the cause had remained unexplained since infancy. He was eventually diagnosed with hyperimmunoglobulinaemia D syndrome (HIDS); one of the autoinflammatory syndromes.
authors.affiliation: Norfolk and Norwich University Hospital NHS Foundation Trust, Norwich, UK ravi.varma@hotmail.co.uk.
authors.forename: Ravi
authors.initials: R
authors.lastname: Varma
authors.affiliation: Norfolk and Norwich University Hospital NHS Foundation Trust, Norwich, UK.
authors.forename: Gary
authors.initials: G
authors.lastname: Campbell
authors.affiliation: Papworth Hospital NHS Foundation Trust, Cambridge, UK.
authors.forename: Helen
authors.initials: H
authors.lastname: Baxendale
mesh_terms: D000328:Adult; D005335:Fever of Unknown Origin / Q000209:etiology*; D006801:Humans; D008297:Male; D054078:Mevalonate Kinase Deficiency; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000235:genetics
chemical_list: D017853:Phosphotransferases (Alcohol Group Acceptor); C021796:mevalonate kinase
keywords: autoinflammatory syndrome; hyperimmunoglobulinaemia D syndrome; periodic fever syndrome; pyrexia of unknown origin