title: [Hereditary intermittant fever].
abstract: Other than familial mediterranean fever: Four hereditary diseases presenting in the form of intermittent inflammatory flares are now recognized and have been characterised clinically and genetically. At the head of this group is Familial Mediterranean Fever (FMF), which affects thousands of patients originating from the Mediterranean area. However the familial Mediterranean Fever is no longer the only recurrent hereditary inflammatory disease. Three other entities have now been clearly defined: intermittent fever secondary to mutations in the type 1A Tumour Necrosis Factor receptor (TNF), of dominant autosomic genetic transmission, the hyperimmunoglobulinemia D syndrome and an entity regrouping the Muckle Wells syndrome, familial cold-induced urticaria, and the Chronic Infantile Neurological Cutaneous and Articular (CINCA) syndrome.
  
  
  CONCLUSIONS
  Because they require specific management and treatment, precise diagnosis of these entities is crucial.
authors.affiliation: Service de médecine interne, Hôtel-Dieu, Assistance publique-Hôpitaux de Paris. gilles.grateau@htd.ap-hop-paris.fr
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
authors.forename: Brigitte
authors.initials: B
authors.lastname: Granel
authors.forename: Véronique
authors.initials: V
authors.lastname: Hentgen
authors.forename: Catherine
authors.initials: C
authors.lastname: Dodé
authors.forename: Laurence
authors.initials: L
authors.lastname: Cuisset
authors.forename: Marc
authors.initials: M
authors.lastname: Delpech
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000235:genetics; D002648:Child; D003080:Cold Temperature; D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology*; D006801:Humans; D007072:Immunoglobulin D; D007223:Infant; D018124:Receptors, Tumor Necrosis Factor / Q000235:genetics; D047888:Receptors, Tumor Necrosis Factor, Type I; D012061:Relapsing Fever / Q000235:genetics* / Q000473:pathology*; D013577:Syndrome; D014581:Urticaria / Q000209:etiology
chemical_list: D015703:Antigens, CD; D007072:Immunoglobulin D; D018124:Receptors, Tumor Necrosis Factor; D047888:Receptors, Tumor Necrosis Factor, Type I