title: Amyloidosis and auto-inflammatory syndromes.
abstract: Amyloidosis remains currently a severe potential complication of many chronic inflammatory disorders. It is not exactly know why some patients develop a progressive amyloidosis, whereas others do not although latent deposits may be present. A permanent acute phase response, ideally evaluated with serial measurement of serum protein SAA, the precursor of the AA protein deposited in tissues, seems to be a prerequisite to the development of inflammatory (AA) amyloidosis. Genetic factors have however been recently emphasized. Among persistent or emerging causes of AA amyloidosis, hereditary periodic fever syndromes also known as auto-inflammatory syndromes are a group of diseases characterised by intermittent bouts of clinical inflammation with focal organ involvement mainly: abdomen, musculoskeletal system and skin. The most frequent is familial Mediterranean fever which affects patients of Mediterranean descent all over the world. Three other types have been recently clinically as well as genetically characterised. A thorough diagnosis is warranted, as clinical and therapeutic management is specific for each of these diseases.
authors.affiliation: Service de médecine interne, L'Hôtel-Dieu, Assistance publique-hôpitaux de Paris, Paris, France. gilles.grateau@tnn.ap-hop-paris.fr
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
authors.forename: Isabelle
authors.initials: I
authors.lastname: Jéru
authors.forename: Saad
authors.initials: S
authors.lastname: Rouaghe
authors.forename: Cécile
authors.initials: C
authors.lastname: Cazeneuve
authors.forename: Nathalie
authors.initials: N
authors.lastname: Ravet
authors.forename: Philippe
authors.initials: P
authors.lastname: Duquesnoy
authors.forename: Laurence
authors.initials: L
authors.lastname: Cuisset
authors.forename: Catherine
authors.initials: C
authors.lastname: Dodé
authors.forename: Marc
authors.initials: M
authors.lastname: Delpech
authors.forename: Serge
authors.initials: S
authors.lastname: Amselem
mesh_terms: D000686:Amyloidosis / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D000818:Animals; D001327:Autoimmune Diseases / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007249:Inflammation / Q000188:drug therapy* / Q000473:pathology