硬皮病的治疗及进展

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櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒櫒24nomaJ.JNatlCancerInst2001931638-1643.25GuntherKLeierJHenningGetal.Predictionoflymphnodeme-tastasisincolorectalcarcinomabyexpressionofchemokinereceptorCCR7J.IntJCancer2005116726-733.26MashinoKSadanagaNYamaguchiHetal.Expressionofchemo-kinereceptorCCR7isassociatedwithlymphnodemetastasisofgas-triccarcinomaJ.CancerRes2002622937-2941.27RichmondAThomasHG.Purificationofmelanomagrowthstimulato-ryactivityJ.JCellPhysiol1986129375-384.28BalentienEMufsonBEShattuckRLetal.EffectsofMGSA/GROalphaonmelanocytetransformationJ.Oncogene199161115-1124.29AnisowiczAMessineoMLeeSWetal.AnNF-kappaB-liketran-scriptionfactormediatesIL-1/TNF-alphainductionofgroinhumanfibroblastsJ.JImmunol1991147520-527.30HaskillSPeaceAMorrisJetal.Identificationofthreerelatedhu-manGROgenesencodingcytokinefunctionsJ.ProcNatlAcadSci1990877732-7736.31ColomboMPMaccalliCMatteiSetal.ExpressionofcytokinegenesincludingIL-6inhumanmalignantmelanomacelllinesJ.MelanomaRes19922181-189.32GravesDTBarnhillRGalanopoulosTetal.Expressionofmonocytechemotacticprotein-1inhumanmelanomainvivoJ.AmJPathol19921409-14.33RobledoMMBartolomeRALongoNetal.ExpressionoffunctionalchemokinereceptorsCXCR3andCXCR4onhumanmelanomacellsJ.JBiolChem200127645098-45105.34PayneASCorneliusLA.TheroleofchemokinesinmelanomatumorgrowthandmetastasisJ.JInvestDermatol2002118915-922.35KawadaKSonoshitaMSakashitaHetal.PivotalroleofCXCR3inmelanomacellmetastasistolymphnodesJ.CancerRes2004644010-4017.36Longo-ImedioMILongoNTrevinoIetal.ClinicalsignificanceofCXCR3andCXCR4expressioninprimarymelanomaJ.IntJCanc-er2005117861-865.37SagerRAnisowiczAPikeMCetal.StructuralregulatoryandfunctionalstudiesoftheGROgeneandproteinJ.Cytokines1992496-116.38Shattuck-BrandtRLRichmondA.EnhanceddegradationofI-kap-paBalphacontributestoendogenousactivationofNF-kappaBinHs294TmelanomacellsJ.CancerRes1997573032-3039.39SchadendorfDMollerAAlgermissenBetal.IL-8producedbyhu-manmalignantmelanomacellsinvitroisanessentialautocrinegrowthfactorJ.JImmunol19931512667-2675.40GutmanMSinghRKXieKetal.Regulationofinterleukin-8ex-pressioninhumanmelanomacellsbytheorganenvironmentJ.CancerRes1995552470-2475.41MoralesJHomeyBVicariAPetal.CTACKaskin-associatedche-mokinethatpreferentiallyattractsskin-homingmemoryTcellsJ.ProcNatlAcadSciUSA19999614470-14475.42KawadaKSonoshitaMSakashitaHetal.PivotalroleofCXCR3inmelanomacellmetastasistolymphnodesJ.CancerRes2004644010-4017.2012-09-03【】。。。510515【】。localizedsclerodermasystemicsclerosisSSc。。。【】【】R751.05【】A【】1672-6170201301-0021-06AdvancesontreatmentofsclerodermaZENGKangLAIKuanCHENPing-jiaoDepartmentofDer-matologyandRheumatologyNanfangHospitalSouthenMedicalUniversityGuangzhou510515China【Abstract】SystemicsclerosisSScisandconnectivetissuediseasecharacterizedbyfibrosisofskinandinternalorgans.Local-izedsclerodermaonlyinvolveskin.Thepathogenesisofthessdiseasesisnotclear.Thoughnewtherapeuticmethodskeepcomingoutandadoptednospecifictreatmentisidentified.Treatmentsandadvancesintherapeuticstudyoflocalizedsclerodermaandsystemicsclerosisarereviewedinthispaper.【Keywords】SclerodermaTreatmentAdvances1∶3。lo-calizedsclerodermamorphealinearscleroderma。guttatemorphea、1220131101plaque-likemorphea、generalizedmorphea、—morphea-lichensclerosisetatrophicusoverlap、PasiniPieriniatrophodermaofPasiniandPierini、morpheaprofunda、bullousmorphea、disablingpanscleroticmorphea。systemicsclerosisSScSSc、、progressivesystemicsclerosisPSSacrosclerodermadiffusesclerodermaCRESTcalci-nosiscutisCRaynaudphenomenonResophagealdysfunctionEsclerodactylyStelangiectasiaT。、3。、1、2、34、90%、。、、、、。、、、、、。11.15~7Photo-therapy。、8、。Nar-rowbandultravioletBNBUVB/1broadbandul-travioletA/ultravioletA-1BBUVA/UVA-1。10~2020~30。9UVA-1。10medium-doseMDUVA1。NBUVBUVA1。1.2、、methotrexateMTXMTX。MTX。Fett11119MTX67MTX。MTX0.3~0.4mg/kg·w15~25mg/w9479%。MTX、。“”“”。1.30.005%120.1%13、。D3、。T、。1.4、、D-、-γ。、A、。MRI。、。2SSc2.1D-、、、。①D-DPA2060。5。。Derk14750mg/d、、。②22201311016mg2/3/61。。③SSc。、、SSc。0.5~1.5mg/d3。SSc、。2.2SSc。、、、、、。15、、、ACEI、16、。2.3、2.3.1SSc、、、、。。、。2.3.2CyclophosphamideCTXCTXT、BCD4/CD8T。CTXSSc。、、17CTXInterstitiallungdiseaseinsystemicsclerosisSSc-ILDCTX1~2mgkg·d1。181824。CTX。、、、19CTX1。。Nannini20MetaCTX。2.3.3mycophenolatemofetilMMFMMFIMPDH21。MMFT、BIIIMPDH。MMFCTX。SScMMF62214.2%1223。524MMF。25dcSScMMF4~6DLCO。2615dcSSCMMF12RodnanmRSS。Meta27MMF、。2.3.4、MTX、A、MTXSScSSc。A、SSc。2g/kgintravenousim-munoglobulinIVIGSSc。2.42.4.1RituximabCD20IgG1、、B。、、。SSc3220131101。2814dcSScRituximab。1。Rituximab。“”。2.4.2-α、-β、-αAnti-TNF-αinfliximab、。29TNF-αetanercept。29infliximabdcSSc。-βTGF-βSSc、、30TGF-βanti-TGF-βantibodySSc。imatinibSSc。IIa、31SSc。2.5322、。。33。2.6hematopoieticstemcelltransplantationHSCTHSCTHSCT。HSCT3435。/3637SScCTX、69%mRss30。17%。5737、、SSc、8.7%92%6HSCTSSc。35%92.2~48.7。Ⅱ38HSCT19dcSSc、CTXantithymocyteglobulinATG58%16%1579%278.9%。343923%mRss、。forcedvitalcapacityFVCDLCO6.04%。1174。、、、Ⅱ40CTXHSCTSSc。。、、、、。3、。、、。【】1PrinzJCKutasiZWeisenseelPetal.Borrelia-associatedearly-onsetmorpheaaparticulartypeofsclerodermainchildhoodandadolescencewithhightiterantinuclearantibodiesResultsofaco-hortanalysi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