SystemicLupus

ErythematosusZhongshanHospital於強內(nèi)科學系統(tǒng)性紅斑狼瘡

WhatisLupus

achronic,relapsing,inflammatory,

andoftenfebrilemultisystemicdisease

ofconnectivetissue,characterized

principallybyinvolvementoftheskin.

Joints,kidneysandserosalmembranes.

anautoimmunedisease

內(nèi)科學系統(tǒng)性紅斑狼瘡

一.Typesoflupus

▲Discoid

▲Systemic

▲Drug-induced內(nèi)科學系統(tǒng)性紅斑狼瘡二.Characteristicofdisease:▲

Atuoimmune▲Targettissuedamage:connectiontissue(shin、Joint、

muscles)▲Predominantlypopulation：

womenduringtheirreproductiveyear90%▲morbidity：70per100，000

內(nèi)科學系統(tǒng)性紅斑狼瘡三.Pathogenicfactor▲Susceptibilitygene：HLA-DR/DQ

▲EnvironmentalFactors：

a.ultravioletlightb.foodhighcaloris,saturatedfat,sproutc.drugsisoniazid,methyldopaD-penicillaminechlorpromazine▲Sexhormone▲Infectionsagents內(nèi)科學系統(tǒng)性紅斑狼瘡

四.pathogenesis

withacomplexsetofimmunologicabnormalitiesthatappeartoinvolvemultiplemechanismsofdysregulation內(nèi)科學系統(tǒng)性紅斑狼瘡Susceptibilitygene

EnvironmentalFactorsSexhormone

HelperTcellactivity

HyperactivatedBcellImmunecomplex

Selfantibody

+selfantigensMultiplesysteminvolvementvasculitis內(nèi)科學系統(tǒng)性紅斑狼瘡五.Pathology

1.vasculitis

2.lupusnephritis

a.minimalchange

b.mesangialproliferative

c.focalsegmentallesions

d.diffuse

e.membranous

f.sclerosing

內(nèi)科學系統(tǒng)性紅斑狼瘡

六.Clinicalmenifestations

ofSLE

-----Multiplesystem

involvement

內(nèi)科學系統(tǒng)性紅斑狼瘡

1.

Nonspecificsymptom

●

fever

●lossofweight

●debilitity內(nèi)科學系統(tǒng)性紅斑狼瘡

2.Skinandmucosa

●skinsymptomsin80%ofpatients

●

specificfeatures:40%

malarrash

discoidrash

●nonspecificfeatures60%

●photosensitivity40%

●baldness40%

●oralulcer60%

●Raynaud’sphenomenon

內(nèi)科學系統(tǒng)性紅斑狼瘡內(nèi)科學系統(tǒng)性紅斑狼瘡內(nèi)科學系統(tǒng)性紅斑狼瘡內(nèi)科學系統(tǒng)性紅斑狼瘡

3.Jointandmuscle80%

●arthritis---nonerosion

●largeandsmalljoints

●myosalgia40%

●myositis5%內(nèi)科學系統(tǒng)性紅斑狼瘡

4.Kidney

▲

renalbiopsycanbefound

renalinvolvementisall

▲

presentinabout70%of

patients

▲

25%patientsaredeadin

causeofrenal

involvement

內(nèi)科學系統(tǒng)性紅斑狼瘡

5.Heart

●pericarditis

pericardialeffusions

●

myocarditis

●

endocarditis

cardiacvalves

vegetation內(nèi)科學系統(tǒng)性紅斑狼瘡

6.Lungs

●

acutelupuspenumonitis3%

●interstitialpneumonia

pulmonaryhypertension

●pleuritis

pleuralfrictionsound

hydrothorax內(nèi)科學系統(tǒng)性紅斑狼瘡interstitialpneumonia內(nèi)科學系統(tǒng)性紅斑狼瘡

7.Neuropsychiatric

involvement

●

CNS

unfavourableprognosis

activity

cranial

nerves

spinalcord

●peripheralnervoussystem

●mentaldisorder

內(nèi)科學系統(tǒng)性紅斑狼瘡

8.Digestivesystem

●

appetite

loss，vomiting

●abdominalpain

diarrhea

ascites

●ALT\AST

●pancreatitis

●intestinalperforation

obstruction

●mesentery

vasculitis

內(nèi)科學系統(tǒng)性紅斑狼瘡

9.Hematologicsystem

●

anemia60%

hemolyticanemia10%

●leukopenia40%

●thrombocytopenia20%

ITP

●lymphadenectasis

內(nèi)科學系統(tǒng)性紅斑狼瘡

10.eye內(nèi)科學系統(tǒng)性紅斑狼瘡

11.OverlapCTD

RA

SS內(nèi)科學系統(tǒng)性紅斑狼瘡

七.Laboratoryfindings

●

ESR

●CRP

內(nèi)科學系統(tǒng)性紅斑狼瘡

Antibody

1.ANA(antinuclearantibody)

screening

test內(nèi)科學系統(tǒng)性紅斑狼瘡

2.Antidouble-stranded

DNAantibody

(ds-DNA)

●

specific

●activity

●strongassociationof

lupusnephritis

內(nèi)科學系統(tǒng)性紅斑狼瘡

3.AntiSmithantibody(Sm)

●

markerantibody

●non-activity內(nèi)科學系統(tǒng)性紅斑狼瘡

4.Antiphospholipidantibody

●

arterial\venousthrombosis

●pregnancymorbidity

●thrombocytopenia

內(nèi)科學系統(tǒng)性紅斑狼瘡

5.AntiSS-A

SS-B

RNP(ribonucleoprotein)

RF內(nèi)科學系統(tǒng)性紅斑狼瘡

6.Complement

●

depressedC3\C4\CH50

●activity內(nèi)科學系統(tǒng)性紅斑狼瘡

7.CT

X-ray

ultrasound內(nèi)科學系統(tǒng)性紅斑狼瘡

八.Diagnosis

The1982RevisedCriteriafor

ClassificationofSLE內(nèi)科學系統(tǒng)性紅斑狼瘡

1.Malarrash

2.Discoidrash

3.Photosensitivity

4.Oralulcers

5.Arthritis

6.Serositis

a)Pleuritisb)Pericarditis

內(nèi)科學系統(tǒng)性紅斑狼瘡

7.Renaldisorder

a)Persistent

proteinuria>0.5g/dor>3+

OR

b)mayberedcell,hemoglobin,

granular,tubular,ormixed

內(nèi)科學系統(tǒng)性紅斑狼瘡

8.Neurologicdisorder

內(nèi)科學系統(tǒng)性紅斑狼瘡

9.Hematologicdisorder

a)Hemolyticanemia

OR

b)Leukopenia<4,000

OR

c)Lyphopenia<1,500

OR

d)Thrombocytopenia<100,000

內(nèi)科學系統(tǒng)性紅斑狼瘡

10.Immunologicdisorder

a)PositiveLEcell

OR

b)Anti-DNA

OR

c)Anti-Sm

OR

d)Falsepositiveserologic

testforsyphilis

內(nèi)科學系統(tǒng)性紅斑狼瘡

11.Antinuclearantibody

內(nèi)科學系統(tǒng)性紅斑狼瘡

apersonshallbesaidtohaveSLEifany4ormoreofthe11

criteriaarepresent,seriallyorsimultaneously,duringanyintervalofobservation內(nèi)科學系統(tǒng)性紅斑狼瘡

九.ManagementofSLE

1.removethecause

drug

food

uvlightinfection

haverest內(nèi)科學系統(tǒng)性紅斑狼瘡

2.Discoid

nonsteroidalanti-

inflam