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1、CurrentDrugTargets-Inflammation&Allergy,2005,4,57-6557AmyloidosisandAuto-InflammatorySyndromesGillesGrateau*,1,2,IsabelleJéru3,SaadRouaghe1,CécileCazeneuve3,NathalieRavet1,PhilippeDuquesnoy3,LaurenceCuisset2,CatherineDodé2,MarcDelpech2andSergeAmselem31Servicedemédecineinterne,L
2、'Hôtel-Dieu,Assistancepublique-hôpitauxdeParis(AP-HP),Paris,France2Equipegénétiqueetphysiopathologiedesmaladiesinflammatoireshéréditaires,Départementdegénétique,développementetpathologiemoléculaire,UnitémixteINSERMU567-CNRSUMR8104,InstitutCochin,Paris,France3GénétiqueMoléculair
3、eetPhysiopathologieINSERMU468,Créteil,FranceAbstract:Amyloidosisremainscurrentlyaseverepotentialcomplicationofmanychronicinflammatorydisorders.Itisnotexactlyknowwhysomepatientsdevelopaprogressiveamyloidosis,whereasothersdonotalthoughlatentdepositsmaybepresent.Apermanentacutepha
4、seresponse,ideallyevaluatedwithserialmeasurementofserumproteinSAA,theprecursoroftheAAproteindepositedintissues,seemstobeaprerequisitetothedevelopmentofinflammatory(AA)amyloidosis.Geneticfactorshavehoweverbeenrecentlyemphasized.AmongpersistentoremergingcausesofAAamyloidosis,here
5、ditaryperiodicfeversyndromesalsoknownasauto-inflammatorysyndromesareagroupofdiseasescharacterisedbyintermittentboutsofclinicalinflammationwithfocalorganinvolvementmainly:abdomen,musculoskeletalsystemandskin.ThemostfrequentisfamilialMediterraneanfeverwhichaffectspatientsofMedite
6、rraneandescentallovertheworld.Threeothertypeshavebeenrecentlyclinicallyaswellasgeneticallycharacterised.Athoroughdiagnosisiswarranted,asclinicalandtherapeuticmanagementisspecificforeachofthesediseases.THEGENERALCONCEPTOFAMYLOIDOSISthediseasesassociatedwithamyloiddeposits.Fourma
7、inentitieswerethusAmyloidwasfirstdescribedinthenineteenthcenturyasanrecognized:primaryamyloidosis(i.e.amyloidosiswithoutanydetectableextracellularmaterialfoundintissuesofpatientsaffectedwithchronicinfectiouscause,secondaryamyloidosis(toaninfectiousdisease),infectiousdiseases[1]
8、.Amyloidhasthenbeenobservedinvariousclinicalhereditary
