系统性红斑狼疮留学生上课.ppt

SystemicLupusErythematosus DoctorNaLiBinzhoumedicalcollegeYantaiaffiliatedhospital SLE Autoimmunediseasethataffectsmultisystems1 5millioncasesoflupusPrevalenceof17to48per100 000populationWomen Men 9 1ratio90 casesarewomenAfricanAmericans WhitesOnsetusuallybetweenagesof15and45years butCanoccurinchildhoodorlaterinlife ClinicalManifestations Forthepurposeofidentifyingpatientsinclinicalstudies apersonhasSLEif4ormoreofthe11criteriaarepresent seriallyorsimultaneously duringanyintervalofobservation specificity95 sensitivity75 ItisimportanttorememberthatapatientmayhaveSLEandnothave4criteria Criteria ButterflyrashDiscoidlupusPhotosensitivityOralulcersArthritisSerositis 7 Neurologicd o8 Hematologicd o9 Renald o10 Immunologic anti DNA anti Sm falseposSTS11 Anti nuclearantibody Cutaneous Mostcommonrashisphotosensitive raisederythematousmalarrash 55 85 developatsomepointindiseaseDiscoidLupusErythematosus DLE 15 30 circular scalyhyperpimentedlesionswitherythematousrim atrophiccenter canbedisfiguringMouth vaginal nasalulcersAlopecia maybediffuseorpatchy Occurs50 MalarRash DiscoidRash OralUlcers MSK Polyarthritis mildtodisabling occursmostfrequentlyinhands wrists knees Occurs90 Jointdeformitiesoccurinonly10 ArthritisofSLEtendstobetransitoryIfsinglejointhaspersistentpain considerosteonecrosis prevalenceincreasedinSLEovergeneralpopulation especiallyifonsteroids MyositiswithelevatedCKandweaknessrarelyoccurs Arthritis Serositis Pulmonary Pleuritiswithorwithouteffusion ifcaseismild tx NSAIDS ifcaseissevere tx steroidsLife threateningmanifestations interstitialinflammationwhichcanleadtofibrosisandintra alveolarhemorrhage AlsopneumothoraxandpulmonaryHTNcanoccur Serositis Cardiac Pericarditis mostcommoncardiacmanifestationandusuallyrespondstoNSAIDs Myocarditis rare andfibrinousendocarditis Libman Sacks mayoccur SteroidsplustreatmentforCHF arrhythmiaorembolicevents MIduetoatherosclerosiscanoccurin 35y o Neuro Cranialorperipheralneuropathyoccursin10 15 itisprobablysecondarytovasculitisinsmallarteriessupplyingnerves DiffuseCNSdysfunction memoryandreasoningdifficultyHeadache ifexcruciating oftenindicateacuteflareSeizuresofanytypePsychosis mustdistinguishfromsteroid inducedpsychosis occursin1stweeksoftxatdoses 40mgprednisoneandresolvesafterseveraldaysofreducingorstoppingtx Cont TIA Stroke mostlyincreasedamongpatientsthatareAPLApositive50 foldincreaseinriskofvasculareventsinwomenunder45comparedtohealthywomenTreatmentforclottingeventislong termanticoagulation Heme Anemia usuallyNormochromic normocyticLeukopenia almostalwaysconsistsoflymphopenia notgranulocytopeniaThrombocytopenia Renal Nephritis usuallyasymptomatic soalwayscheckUAifpatienthasknownorsuspectedSLEOccursearlyincourseofdisease ifnotpresentw in1yr probablywillnotoccur Histologicclassificationbyrenalbiopsyisusefultoplantherapy HistologicClassifications ClassIisminimalmesangialglomerulonephritiswhichishistologicallynormalonlightmicroscopybutwithmesangialdepositsonelectronmicroscopy ClassIIisbasedonafindingofmesangialproliferativelupusnephritis Thisformtypicallyrespondscompletelytotreatmentwithcorticosteroids ClassIIIisfocalproliferativenephritisandoftensuccessfullyrespondstotreatmentwithhighdosesofcorticosteroids ClassIVisdiffuseproliferativenephritis Thisformismainlytreatedwithcorticosteroidsandimmunosuppressantdrugs ClassVismembranousnephritisandischaracterizedbyextremeedemaandproteinloss ClassVIGlomerulosclerosis Immunoglobulins Anti dsDNAIgG veryspecific maycorrelatewithdiseaseactivityAnti Sm specific butonlypresentin25 ofcases doesnotcorrelatewithactivityAPLA notspecific Usedtoidentifypatientsatincreasedriskforclots thrombocytopeniaandfetalloss ANA ANA 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 RimandHomogenous Additionalwork up Serumcr andalbuminCBCw diffU AESRComplementlevelsRenalbxifwarranted Treatment Treatmentplansarebasedonpatientage sex health symptoms andlifestyleGoalsoftreatmentareto preventflares treatflareswhentheyoccur minimizeorgandamageandcomplications Conservativemanagement Forthosew outmajororganinvolvement NSAIDs tocontrolpain swelling andfeverCautionw NSAIDSthough SLEptsareatincreasedriskforasepticmeningitisAntimalarials Generallytotreatfatiguejointpain skinrashes andinflammationofthelungsCommonlyused HydroxycholorquineUsedaloneorincombinationwithotherdrugs Cont Corticosteroids MainstayofSLEtreatment TorapidlysuppressinflammationUsuallystartwithhigh doseIVpulseandconverttoPOsteroidswithgoaloftaperingandconvertingtosomethingelse Commonlyused prednisone hydrocortisone methylprednisolone anddexamethasone Immunosuppressives PrimarilyforCNS renalinvolvementMycophenolatemofetil cellcept Azathioprine imuran requiresseveralmonthstobeeffective effec