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Systemic Lupus

Erythematosus

Justin A. CrockerAM Report 12/2/09

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SLE

Autoimmune disease that aects mu!tisystems 1." mi!!ion cases o !upus

#re$a!ence o 1% to &' per 100(000 popu!ation )omen * Men + 9,1 ratio 90- cases are omen Arican Americans * )hites

nset usua!!y eteen aes o 1" and &" years(ut

Can occur in chi!dhood or !ater in !ie

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C!inica! Maniestations

or the purpose o identiyin patients inc!inica! studies( a person has SLE i & or

more o the 11 criteria are present( seria!!yor simu!taneous!y( durin any inter$a! ooser$ation. 3speciicity 9"-( sensiti$ity%"-4

5t is important to rememer that a patientmay ha$e SLE and not ha$e & criteria.

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Criteria

1. 6utter!y rash

2. 7iscoid !upus

8. #hotosensiti$ity

&. ra! u!cers

". Arthritis

. Serositis

%. :euro!oic d/o

'. ;emato!oic d/o

9. Rena! d/o

10.5mmuno!oic, anti+

7:A( anti+Sm( a!se

pos S

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Cutaneous

Most common rash is photosensiti$e( raisederythematous ma!ar rash. ""+'"- de$e!op atsome point in disease

7iscoid Lupus Erythematosus 37LE4, 1"+80-circu!ar( sca!y hyperpimented !esions itherythematous rim( atrophic center=can edisiurin

Mouth/$aina!/nasa! u!cers A!opecia, may e diuse or patchy. ccurs "0-

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Ma!ar Rash

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7iscoid Rash

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ra! >!cers

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MS?

#o!yarthritis( mi!d to disa!in( occurs mostre@uent!y in hands( rists( knees. ccurs 90-

Joint deormities occur in on!y 10- Arthritis o SLE tends to e transitory 5 sin!e oint has persistent pain( consider

osteonecrosis 3pre$a!ence increased in SLE o$erenera! popu!ation( especia!!y i on steroids.4

Myositis ith e!e$ated C? and eakness rare!yoccurs

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Arthritis

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Serositis + #u!monary

#!euritis ith or ithout eusion

+ i case is mi!d( tB, :SA57S

+ i case is se$ere( tB, steroids Lie+threatenin maniestations, interstitia!

in!ammation hich can !ead to irosis and

intra+a!$eo!ar hemorrhae. A!so pneumothoraB and pu!monary ;

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Serositis Cardiac

#ericarditis, most common cardiac maniestation

and usua!!y responds to :SA57s.

Myocarditis 3rare4 and irinous endocarditis3Liman+Sacks4 may occur. Steroids p!us

treatment or C;/arrhythmia or emo!ic e$ents.

M5 due to atherosc!erosis can occur in D8" y/o

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:euro

Crania! or periphera! neuropathy occurs in 10+1"-( it isproa!y secondary to $ascu!itis in sma!! arteriessupp!yin ner$es.

7iuse C:S dysunction, memory and reasonindiicu!ty ;eadache, i eBcruciatin( oten indicate acute !are Seiures o any type #sychosis, must distinuish rom steroid+induced

psychosis 3occurs in 1steeks o tB at doses F&0mprednisone and reso!$es ater se$era! days o reducinor stoppin tB4

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Cont.

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;eme

Anemia, usua!!y :ormochromic(

normocytic

Leukopenia, a!most a!ays consists o!ymphopenia( not ranu!ocytopenia

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Rena!

:ephritis, usua!!y asymptomatic( so

a!ays check >A i patient has knon or

suspected SLE ccurs ear!y in course o disease+i not

present /in 1 yr( proa!y i!! not occur.

;isto!oic c!assiication y rena! iopsy isuseu! to p!an therapy

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;isto!oic C!assiications C!ass 5 is minima! mesania! !omeru!onephritis hich is

histo!oica!!y norma! on !iht microscopy ut ith mesania!deposits on e!ectron microscopy.

C!ass 55 is ased on a indin o mesania! pro!ierati$e !upusnephritis.

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5mmuno!ou!ins

Anti+ds7:A 5H, $ery speciic( may

corre!ate ith disease acti$ity

Anti+Sm, speciic( ut on!y present in 2"-o cases( does not corre!ate ith acti$ity

A#LA, not speciic. >sed to identiy

patients at increased risk or c!ots(thromocytopenia and eta! !oss

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A:A

A:A, positi$e in 9"- o cases. #retest

proai!ity aects interpretation. 5n #C# settin(

2- or SLE. 5n rheum, 80- Lo #ositi$e 31,10 or !oer4, SLE !ike!ihood

D2- 3D2- or rheumato!oists4

;ih #ositi$e 31,820 or hiher4, SLE !ike!ihood,

2+1%- 382+'1- or rheumato!oists4

SLE speciic patterns, Rim and ;omoenous

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Additiona! ork+up

+ Serum cr. and a!umin

+ C6C / di

+ >/A

+ ESR

+

Comp!ement !e$e!s+ Rena! B i arranted

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Conser$ati$e manaement

or those /out maor oran in$o!$ement. :SA57s, to contro! pain( se!!in( and e$er

Caution / :SA57S thouh. SLE pts are atincreased risk or aseptic meninitis

Antima!aria!s, Henera!!y to treat atiue ointpain( skin rashes( and in!ammation o the !uns

Common!y used, ;ydroBycho!or@uine >sed a!one or in comination ith other drus

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Cont.

Corticosteroids 3Mainstay o SLE treatment4

sua!!y start ith hih+dose 5G pu!se and con$ertto # steroids ith oa! o taperin and

con$ertin to somethin e!se.

Common!y used, prednisone( hydrocortisone(methy!predniso!one( and deBamethasone

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5mmunosuppressi$es

#rimari!y or C:S/rena! in$o!$ement Mycopheno!ate moeti! 3ce!!cept4 Aathioprine 3imuran4, re@uires se$era! months to e

eecti$e( eecti$e in sma!!er percentae o patients M