内科学系统性红斑狼疮於强课件

内科学系统性红斑狼疮於强36、如果我们国家的法律中只有某种神灵，而不是殚精竭虑将神灵揉进宪法，总体上来说，法律就会更好。——马克·吐温37、纲纪废弃之日，便是暴政兴起之时。——威·皮物特38、若是没有公众舆论的支持，法律是丝毫没有力量的。——菲力普斯39、一个判例造出另一个判例，它们迅速累聚，进而变成法律。——朱尼厄斯40、人类法律，事物有规律，这是不容忽视的。——爱献生1Systemic

LupusErythematosus2Zhongshan

Hospital於强What

is

Lupus3a

chronic,

relapsing,

inflammatory,and

often

febrile

multisystemic

diseaseof

connective

tissue,

characterizedprincipally

by

involvement

of

the

skin.Joints,

kidneys

and

serosal

membranes.an

autoimmune

disease一.Types

of

lupus4▲

Discoid▲

Systemic▲

Drug-induced二.

Characteristic

of

disease:5▲

Atuoimmune▲

Target tissue

damage:connectiontissue

(shin、Joint、muscles)▲

Predominantly

population：womenduring their

reproductive

year

90%▲

morbidity：70

per

100，000三.

Pathogenic

factor6▲Susceptibility gene：HLA-DR/DQ▲Environmental

Factors：a.

ultraviolet

lightb.

foodc.

drugshigh

caloris,saturated

fat,sproutisoniazid,methyldopaD-penicillaminechlorpromazine▲

Sex

hormone▲

Infections

agents四.pathogenesis7with

a

complex

set

ofimmunologic

abnormalities

thatappear

to

involve

multiplemechanisms

of

dysregulationSusceptibility

geneEnvironmental

Factors Sex

hormoneHelper T

cell

activityHyperactivated

B

cellImmune

complexSelf

antibody+

self

antigensMultiple

system

involvementvasculitis8五.Pathology9vasculitislupus

nephritisminimal

changemesangial

proliferativefocal

segmental

lesionsdiffusemembranoussclerosing六.Clinical

menifestationsof

SLE10-----MultiplesysteminvolvementNonspecific

symptomfeverloss

of

weightdebilitity112.Skin

and

mucosa12skin

symptoms

in

80%

of

patientsspecific features:

40%malar

rashdiscoid

rashnonspecific features

60%photosensitivity

40%baldness

40%oral

ulcer

60%Raynaud’s

phenomenon1314153.

Joint and

muscle

80%16arthritis---non

erosionlarge

and

small

jointsmyosalgia

40%myositis

5%4.

Kidney17▲

renal

biopsy

can

be

foundrenal

involvement

is

all▲

present

in

about

70%

ofpatients▲

25%

patients

are

deadincause

of

renalinvolvement5.

Heart18pericarditispericardial

effusionsmyocarditisendocarditiscardiac

valvesvegetation6.

Lungs193%acute lupus

penumonitisinterstitial

pneumoniapulmonary

hypertensionpleuritispleural

friction

soundhydrothoraxinterstitial

pneumonia207.Neuropsychiatricinvolvement21CNSunfavourable

prognosisactivitycranial

nervesspinal

cordperipheral

nervous

systemmental

disorder8.Digestive

system22appetite loss，

vomitingabdominal

paindiarrheaascitesALT\ASTpancreatitisintestinal

perforation obstructionmesentery

vasculitis9.Hematologic

system23anemia

60%hemolytic

anemia

10%leukopenia

40%thrombocytopenia

20% ITPlymphadenectasis10.eye2411.Overlap

CTDRASS25findings26七.LaboratoryESRCRPAntibody1.

ANA

(antinuclear

antibody)screening

test272.

Anti

double-strandedDNA

antibody28(ds-DNA)specificactivitystrong

association

of lupus

nephritisAnti

Smith

antibody(Sm)marker

antibodynon-activity294.Antiphospholipid

antibody30arterial\venous

thrombosispregnancy

morbiditythrombocytopenia5.

Anti

SS-ASS-BRNP(ribonucleoprotein)RF316.

Complement32C3\C4\CH50depressedactivity7.

CTX-rayultrasound33八.DiagnosisThe

1982

Revised

Criteria

forClassification

of

SLE341.

Malar

rash35Discoid

rashPhotosensitivityOral

ulcersArthritisSerositisa)

Pleuritis

b)

Pericarditis7.

Renal

disorder36Persistentproteinuria

>

0.5

g/d or

>

3+ORmay

be

red

cell,hemoglobin,granular,

tubular,

or

mixed8.

Neurologic

disorder379.

Hematologic

disorder38Hemolytic

anemiaORLeukopenia

<

4,000ORLyphopenia

<

1,500ORThrombocytopenia

<100,00010.

Immunologic

disorder39Positive

LEcellORAnti-DNAORAnti-SmORFalse

positive

serologictest forsyphilis11.

Antinuclear

antibody40a

person

shall

be

said

tohaveSLE if

any

4

or

more

of

the

11criteria

are

present,

seriallyorsimultaneously,

during

anyinterval

ofobservation41九.Management

of

SLE421.

remove

the

causedrugfooduv

light

infectionhave

rest2.

Discoid43nonsteroidal

anti-inflammatory

drugs（NSAIDs）+antimalarials

(chloroquine）ORlocal steroids

orlow dose

GC3.

SLE44glucocorticoid

(GC)+cytotoxic

drugs(cyclophosphamide)moderate

dose

GC 1mg/kg/d4.lymphocyte-specific cytotoxic

drugintravenous

gamma globulinimmunoablation

with autologous

stem

cell transplantation45十.Prognosis46has

improveddeath

is

caused

most frequen