系统性红斑狼疮-课件(同名128)

SystemicLupusErythematosusSystemicLupusErythematosus1SLEAutoimmunediseasethataffectsmultisystems1.5millioncasesoflupusPrevalenceof17to48per100,000populationWomen>Men-9:1ratio90%casesarewomenAfricanAmericans>WhitesOnsetusuallybetweenagesof15and45years,butCanoccurinchildhoodorlaterinlifeSLEAutoimmunediseasethataff2ClinicalManifestationsForthepurposeofidentifyingpatientsinclinicalstudies,apersonhasSLEif4ormoreofthe11criteriaarepresent,seriallyorsimultaneously,duringanyintervalofobservation.(specificity95%,sensitivity75%)ItisimportanttorememberthatapatientmayhaveSLEandnothave4criteria.ClinicalManifestationsForth3CriteriaButterflyrashDiscoidlupusPhotosensitivityOralulcersArthritisSerositis7.Neurologicd/o8.Hematologicd/o9.Renald/o10.Immunologic:anti-DNA,anti-Sm,falseposSTS11.Anti-nuclearantibodyCriteriaButterflyrash7.Neuro4CutaneousMostcommonrashisphotosensitive,raisederythematousmalarrash.55-85%developatsomepointindiseaseDiscoidLupusErythematosus(DLE):15-30%circular,scalyhyperpimentedlesionswitherythematousrim,atrophiccenter—canbedisfiguringMouth/vaginal/nasalulcersAlopecia:maybediffuseorpatchy.Occurs50%

CutaneousMostcommonrashisp5

MalarRash

MalarRash

6DiscoidRashDiscoidRash7OralUlcersOralUlcers8MSKPolyarthritis,mildtodisabling,occursmostfrequentlyinhands,wrists,knees.Occurs90%Jointdeformitiesoccurinonly10%ArthritisofSLEtendstobetransitoryIfsinglejointhaspersistentpain,considerosteonecrosis(prevalenceincreasedinSLEovergeneralpopulation,especiallyifonsteroids.)MyositiswithelevatedCKandweaknessrarelyoccursMSKPolyarthritis,mildtodisa9ArthritisArthritis10Serositis-PulmonaryPleuritiswithorwithouteffusion -ifcaseismild,tx:NSAIDS -ifcaseissevere,tx:steroidsLife-threateningmanifestations:interstitialinflammationwhichcanleadtofibrosisandintra-alveolarhemorrhage.AlsopneumothoraxandpulmonaryHTNcanoccurSerositis-PulmonaryPleuritis11Serositis–CardiacPericarditis:mostcommoncardiacmanifestationandusuallyrespondstoNSAIDs.Myocarditis(rare)andfibrinousendocarditis(Libman-Sacks)mayoccur.SteroidsplustreatmentforCHF/arrhythmiaorembolicevents.MIduetoatherosclerosiscanoccurin<35y/oSerositis–CardiacPericarditi12NeuroCranialorperipheralneuropathyoccursin10-15%,itisprobablysecondarytovasculitisinsmallarteriessupplyingnerves.DiffuseCNSdysfunction:memoryandreasoningdifficultyHeadache:ifexcruciating,oftenindicateacuteflareSeizuresofanytypePsychosis:mustdistinguishfromsteroid-inducedpsychosis(occursin1stweeksoftxatdoses≥40mgprednisoneandresolvesafterseveraldaysofreducingorstoppingtx)NeuroCranialorperipheralneu13Cont.TIA,Stroke:mostlyincreasedamongpatientsthatareAPLApositive50-foldincreaseinriskofvasculareventsinwomenunder45comparedtohealthywomenTreatmentforclottingeventislong-termanticoagulationCont.TIA,Stroke:mostlyincre14HemeAnemia:usuallyNormochromic,normocyticLeukopenia:almostalwaysconsistsoflymphopenia,notgranulocytopeniaThrombocytopeniaHemeAnemia:usuallyNormochrom15RenalNephritis:usuallyasymptomatic,soalwayscheckUAifpatienthasknownorsuspectedSLEOccursearlyincourseofdisease-ifnotpresentw/in1yr,probablywillnotoccur.HistologicclassificationbyrenalbiopsyisusefultoplantherapyRenalNephritis:usuallyasympt16HistologicClassificationsClassIisminimalmesangialglomerulonephritiswhichishistologicallynormalonlightmicroscopybutwithmesangialdepositsonelectronmicroscopy.ClassIIisbasedonafindingofmesangialproliferativelupusnephritis.Thisformtypicallyrespondscompletelytotreatmentwithcorticosteroids.ClassIIIisfocalproliferativenephritisandoftensuccessfullyrespondstotreatmentwithhighdosesofcorticosteroids.ClassIVisdiffuseproliferativenephritis.Thisformismainlytreatedwithcorticosteroidsandimmunosuppressantdrugs.ClassVismembranousnephritisandischaracterizedbyextremeedemaandproteinloss.ClassVIGlomerulosclerosisHistologicClassificationsClas17ImmunoglobulinsAnti-dsDNAIgG:veryspecific,maycorrelatewithdiseaseactivityAnti-Sm:specific,butonlypresentin25%ofcases,doesnotcorrelatewithactivityAPLA:notspecific.Usedtoidentifypatientsatincreasedriskforclots,thrombocytopeniaandfetallossImmunoglobulinsAnti-dsDNAIgG:18ANAANA:positivein95%ofcases.Pretestprobabilityaffectsinterpretation.InPCPsetting,2%forSLE.Inrheum:30%LowPositive(1:160orlower):SLElikelihood<2%(<26%forrheumatologists)HighPositive(1:320orhigher):SLElikelihood:2-17%(32-81%forrheumatologists)SLEspecificpatterns:RimandHomogenousANAANA:positivein95%ofcas19Additionalwork-upSerumcr.andalbuminCBCw/diffU/AESRComplementlevelsRenalbxifwarrantedAdditionalwork-upSerumcr.an20TreatmentTreatmentplansarebasedonpatientage,sex,health,symptoms,andlifestyleGoalsoftreatmentareto:-preventflares-treatflareswhentheyoccur-minimizeorgandamageandcomplicationsTreatmentTreatmentplansareb21Conservativemanagement Forthosew/outmajororganinvolvement.NSAIDs:tocontrolpain,swelling,andfeverCautionw/NSAIDSthough.SLEptsareatincreasedriskforasepticmeningitisAntimalarials:Generallytotreatfatiguejointpain,skinrashes,andinflammationofthelungsCommonlyused:HydroxycholorquineUsedaloneorincombinationwithotherdrugsConservativemanagement Forth22Cont.Corticosteroids(MainstayofSLEtreatment)TorapidlysuppressinflammationUsuallystartwithhigh-doseIVpulseandconverttoPOsteroidswithgoaloftaperingand