Systemic Lupus Erythematosus qstation.org.ppt

systemiclupuserythematosus definition lupusisaconditionofchronicinflammationcausedbyanautoimmunediseaselupuscanaffectmanypartsofthebody includingthejoints skin kidneys heart lungs bloodvessels andbrain definition whenonlytheskinisinvolved theconditioniscalleddiscoidlupus wheninternalorgansareinvolved theconditioniscalledsystemiclupuserythematosus sle othertypesoflupus subacutecutaneouslupuserythematosusreferstoskinlesionsthatappearonpartsofthebodyexposedtosun thelesionsdonotcausescarring neonatallupusisararediseasethatcanoccurinnewbornbabiesofwomenwithsleorsj gren ssyndromeitissuspectedthatneonatallupusiscausedbyautoantibodiesinthemother sbloodcalledanti ro ssa andanti la ssb othertypesoflupus drug inducedlupushydralazinequinidineprocainamidedilantinisoniazided penicillamine whogetsit slearemorecommoninwomenthanmen abouteighttimesmorecommon lupusisthreetimesmorecommoninafricanamericanwomenthanincaucasianwomencanaffectallagesbutmostcommonlybeginsfromage20to45yearsitismorefrequentinafrican americans nativeamerican andasianslupuscanruninfamilies description atpresent thereisnocureforlupushowever lupuscanbeeffectivelytreatedwithdrugs andmostpeoplewiththediseasecanleadactive healthylives clinicalmanifestation patientswithsleappeartobeathighriskforcoronaryarterydisease infections especiallyoftherespiratoryandurinarysystems alsoarecommoninpatientswiththediseaseandaredifficulttodistinguishfromflaresoflupusactivity morbidityandmortality inonecohortstudyitisfoundthatwithinsevenyearsofdiagnosis 61percentofpatientsdevelopedclinicallydetectableorgandamage withneuropsychiatric 20 5percent musculoskeletal 18 5percent andrenal 15 5percent organsystemsmostcommonlyaffected morbidityandmortality sincethe1950s thefive yearsurvivalrateforpatientswithsystemiclupuserythematosushasincreasedfrom50percenttoarangeof91to97percent highermortalityratesareassociatedwithseizures lupusnephritis andazotemia clinicalfeatures malarrash discoidrash discoidlesionsarethick scarring usuallyraisedorflat red withwell definedborders whichappearonsun exposedsurfaces butinrarecasescanbefoundonnon sun exposedareas theygenerallydonotitch whatcauseslupus theprecisereasonfortheabnormalautoimmunitythatcauseslupusisnotknowninheritedgenes viruses ultravioletlight anddrugsmayallplaysomeroleitalsoisknownthatsomewomenwithslecanexperienceworseningoftheirsymptomspriortotheirmenstrualperiods thisphenomenon togetherwiththefemalepredominanceofsle suggestthatfemalehormonesplayanimportantroleintheexpressionofsle diagnosis nosingletestestablishesthediagnosisofsystemiclupusitisbasedonclinicalandlaboratorycriteriathemostusefullabtestistheantinuclearantibody ana theamericancollegeofrheumatology acr recommendsanatestinginpatientswhohavetwoormoreunexplainedsignsorsymptomslistedintheearlierslide definition classificationcriteriaforsle ana elevationoftheantinuclearantibody ana titerto1 40orhigheristhemostsensitiveoftheacrdiagnosticcriteria theanavaluehastypical peripheral patternmorethan99percentofpatientswithsystemiclupuserythematosushaveanelevatedanatiteratsomepointalthoughasignificantproportionofpatientsmayhaveanegativeanatiterearlyinthedisease diagnosis whentheanaispositive ananaprofileincludingananti double stranddna ananti sm anti smith antibodytest anti ssaandanti ssbisdone boththeanti ds dnaandanti smantibodytestare100 specificforsle ie theyareonlypositiveinpatientsinpatientswithsle diagnosis soinworkingupacaseofpossiblesle firstdoanana iftheanaispositive doananaprofile iftheanaisnegativeandclinicalsignsstronglysuggestsle thencheckforanti ssa roantibodies ifthistestispositve thepatientprobablyhas ana negative sle rare diagnosis treatment treatment treatment treatment treatment patientswithcentralnervoussystemmanifestationsoflupuserythematosuswhopresentwithstatusepilepticus organicbrainsyndromeorcomacanbetreatedwithintravenousmethylprednisolonepulsetherapy patientswithsevereorresistantsymptomsmayalsorequiretreatmentwithintravenouscyclophosphamideand orplasmapheresis however itisusuallynecessarytoruleoutotherconditionsthatmaymimiccentralnervoussystemmanifestationsofsystemiclupuserythematosus includinginfectionandtoxicmetabolicstates treatment patientswithsystemiclupuserythematosushavehigherlevelsofhomocysteine aknownriskfactorforatherosclerosis intervention intheformofbothlifestylemodificationsandpharmacologictherapy folate maybeappropriateinpatientswithsle treatment antiphospholipidantibodysyndromeisoneofthemostcommoncausesofacquiredhypercoagulabilityinthegeneralpopulationandismuchmorecommoninpatientswithsystemiclupuserythematosuspatientswhohavehadvenousorarterialthrombosisappeartobenefitfrommaintenancetherapywithhigh intensity internationalnormalizedratioof3to4 warfarin coumadin insummary systemiclupuserythematosus sle isanautoimmunedisease sleischaracterizedbytheproductionofunusualantibodiesintheblood sleismorecommoninwomenthanmen thecause s ofsleis are unknown however heredity viruses ultravioletlight anddrugsallmayplaysomerole upto10 ofpatientswithsleisolatedtotheskinwilldevelopthesystemicformoflupus sle 4outof11criteriahelpinthediagnosesle treatmentofsleisdirectedtowarddecreasinginflammationand orthelevelofautoimmuneactivity patientswithslecanprevent flares ofdiseasebyavoidingsunexposureandnotabruptlydiscontinuingmedications questions whatisthemostsensitivetestforsle a anti double strandeddnaantibody b anti smith antibody