[Hereditary intermittant fever].
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.
Grateau
Gilles
G
Service de médecine interne, Hôtel-Dieu, Assistance publique-Hôpitaux de Paris. gilles.grateau@htd.ap-hop-paris.fr
Granel
Brigitte
B
Hentgen
Véronique
V
Dodé
Catherine
C
Cuisset
Laurence
L
Delpech
Marc
M
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
D015703:Antigens, CD; D007072:Immunoglobulin D; D018124:Receptors, Tumor Necrosis Factor; D047888:Receptors, Tumor Necrosis Factor, Type I