内科学系统性红斑狼疮於强

SystemicLupus

ErythematosusZhongshanHospital於强achronic,relapsing,inflammatory,

andoftenfebrilemultisystemicdisease

ofconnectivetissue,characterized

principallybyinvolvementoftheskin.WhatisLupus01anautoimmunediseaseJoints,kidneysandserosalmembranes.02

一.Typesoflupus

▲Discoid

▲Systemic

▲Drug-inducedAtuoimmune1Targettissuedamage:2connectiontissue(shin、Joint、3muscles)4Predominantlypopulation：5women6duringtheirreproductiveyear90%7morbidity：70per100，0008二.Characteristicofdisease:三.Pathogenicfactor▲Susceptibilitygene：HLA-DR/DQ

▲EnvironmentalFactors：

a.ultravioletlightb.foodhighcaloris,saturatedfat,sproutc.drugsisoniazid,methyldopaD-penicillaminechlorpromazine▲Sexhormone▲Infectionsagents01020304withacomplexsetofimmunologicabnormalitiesthatappeartoinvolvemultiplemechanismsofdysregulation四.pathogenesisEnvironmentalFactorsSexhormone01HelperTcellactivity02HyperactivatedBcell03Immunecomplex04Selfantibody+selfantigens05Multiplesysteminvolvementvasculitis06Susceptibilitygene五.Pathology

1.vasculitis

2.lupusnephritis

a.minimalchange

b.mesangialproliferative

c.focalsegmentallesions

d.diffuse

e.membranous

f.sclerosingClinicalmenifestations0101020304ofSLE----Multiplesysteminvolvement020304Nonspecificsymptomlossofweightfeverdebilitity

2.Skinandmucosa

●skinsymptomsin80%ofpatients

●

specificfeatures:40%

malarrash

discoidrash

●nonspecificfeatures60%

●photosensitivity40%

●baldness40%

●oralulcer60%

●Raynaud’sphenomenon

01Jointandmuscle80%02arthritis---nonerosion03largeandsmalljoints04myosalgia40%05myositis5%renalbiopsycanbefound1renalinvolvementisall2presentinabout70%of3patients425%patientsaredeadin5causeofrenal6involvement74.KidneyHeartpericarditispericardialeffusionsmyocarditisendocarditiscardiacvalvesvegetation020304050601interstitialpneumoniaacutelupuspenumonitis3%pulmonaryhypertensionhydrothoraxpleuritispleuralfrictionsound6.Lungsinterstitialpneumonia01Neuropsychiatric02involvement03CNS04unfavourableprognosis05activity06cranialnerves07spinalcord08peripheralnervoussystem09mentaldisorder

8.Digestivesystem

●

appetite

loss，vomiting

●abdominalpain

diarrhea

ascites

●ALT\AST

●pancreatitis

●intestinalperforation

obstruction

●mesentery

vasculitis

Hematologicsystem01anemia60%02hemolyticanemia10%03leukopenia40%04thrombocytopenia20%05ITP06lymphadenectasis0710.eyePART1OverlapCTDRASS七.Laboratoryfindings

ESR

CRPAntibodyscreeningtestANA(antinuclearantibody)Antidouble-strandedDNAantibody(ds-DNA)specificactivitystrongassociationoflupusnephritisAntiSmithantibody(Sm)1markerantibody2non-activity3Antiphospholipidantibodyarterial\venousthrombosispregnancymorbiditythrombocytopeniaAntiSS-A01SS-B02RNP(ribonucleoprotein)03RF04ComplementdepressedC3\C4\CH50activity010203X-rayultrasoundCT八.DiagnosisThe1982RevisedCriteriaforClassificationofSLEMalarrash

Discoidrash

Photosensitivity

Oralulcers

Arthritis

Serositis

Pleuritisb)PericarditisRenaldisorder

PersistentOR

mayberedcell,hemoglobin,proteinuria>0.5g/dor>3+granular,tubular,ormixed8.NeurologicdisorderHemolyticanemia9.Hematologicdisorder01Leukopenia<4,000OR02Thrombocytopenia<100,000OR04Lyphopenia<1,500OR03

10.Immunologicdisorder

a)PositiveLEcell

OR

b)Anti-DNA

OR

c)Anti-Sm

OR

d)Falsepositiveserologic

testforsyphilis

AntinuclearantibodyapersonshallbesaidtohaveSLEifany4ormoreofthe11criteriaarepresent,seriallyorsimultaneously,duringanyintervalofobservationManagementofSLE01removethecause02drug03food04