3. Sulchan - Prof. Ssystemic Lupus Erythematosus in Pregnancy Revisi1

8/9/2019 3. Sulchan - Prof. Ssystemic Lupus Erythematosus in Pregnancy Revisi1

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HM Sulchan SofoewanDivision of Fetomaternal Departement ofObstetrics and Gynecology Faculty ofMedicine Gadjah Mada University


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INTRODUCTION

Lupus is heterogenous syndrome with a complexpathogenesis

!ts genetic contribution is clustered on the "# and$p chromosome

!mmune system abnormalities include overactive% lymphocyte that are responsible forautoantibody production

&hese result in tissue and cellular damage whenautoantibodies or immune complexes are directedat one or more cellular nuclear components

'lmost ()* of cases are in women+ theprevalence in women of childbearing age is about" in ,))

&en and -) yrs survival rates are .,* and ,)* !nfection+ lupus /are+ end0organ failure and

cardiovascular disease account for most death


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KINDS OF LUPUS

1L23 is the form of the disease that most peopleare referring to when they say 4lupus5 systemiccan a6ects many part of body

Discord lupus erythematosus3 is a chronic s7in

disorder in which a red+ raised rash appear on theface scalp or elsewhere 1ubacute cutaneous lupus erythematosus3 refers

to s7in lesion that appear or parts of the bodyexposed to sun

Drug induced lupus3 is a form of lupus caused bymedication 8eonatal lupus3 is a rare disease that can occurs

in newborn babies of women with 1L2+ caused byautoantibodies in the mother blood called anti09o:11'; or anti0La:11%;


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THE CAUSES OF LUPUS

Lupus is a complex disease+ its cause iaun7nown

!t is li7ely that a combination of genetic+

environment and possibly hormonal factorswor7 together Genetics plays an important role+ however+

no speci


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' healthy immune system produces protein calledantibodies and speci


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CLINICAL SYMPTOMES

" >ainful or swollen joints and muscle pain - Unexplained fever ? 9ed rashes+ most commonly on the face

@ Ahest pain upon deep breathing , Unusual loss of hair $ >ale or purple


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THE SYSTEMS CAN BE

AFFECTED" idney3 in/ammation :nephritis;

- Lungs3 pleuritis+ in/ammation of the chest cavity+or pneumoni

? Aentral 8ervous 1ystem3 a6ect the brain+headaches+ diEEiness+ memory disturbances+ visionproblem+ seiEures+ stro7e or changes in behavior=

@ %lood vessels3 vasculitis+ a6ecting blood circulation

, %lood3 anemia+ leu7openia+ thrombocytopenia

$ eart3 myocarditis+ endocarditis+ pericarditis+ andthe ris7 of atherosclerosis


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LUPUS AND MATERNAL

OUTCOME !mportant factor3 whether disease is active at

the beginning of pregnancy+ age+ parity Aoexistence of other medical or obstetrical

disorders hether there are an '>' During pregnancy "C? lupus improveH "C?

remains unchangedH "C? worsens !s any given pregnancy+ the chronical

condition can worsen+ or /are without warning .* ris7 of major morbidity pregnancy+ " in -)

chance of a life threating event Generally+ there are due to renal impairemnt+

myocarditis or serositis


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ADJUSTED OR FOR PREDICTION OF

PREGNANCY LOSS

Adjusted OR 95% CI P

Proteinuria

APS

Throbocytpenia

Hypertension

2,

!,"

","

!,#

#,5 $ ,

, $ #,5

," && !,"

, && ,5

'!#

'#!

'#

,#"


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PREGNANCY OUTCOME IS BETTER

IF

" Lupus activity has been #uiescentfor at least $ months beforeconception

- &her is no active renal involvementmanifest by proteinuria or renaldysfunction

? 1uperimposed preec7lampsia doesnot develop

@ &her is no evidence of '>' activity


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EFFECTS OF LUPUS ON THE

FETUS

FG9 and perinatal mortality and morbidityare increased signirognosis is worsened with a lupus /are+

proteinuria+ renal impairment+ hypertensionand preec7lampsia

'dverse fetal caused by decidualvasculopathy with placental infarction anddecrease perfusion

'nti 11':9o; and anti 11%:La; antibodiesmay damage the fetal heart and conductionsystem+ causing neonatal death


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EFFECTS OF LUPUS ON THE

NEONATE

&his unusual syndrome ischaracteriEed by s7in lesion or lupusdermatitis+ hematological and

systemic derangements andoccasionally congenital heart bloc7

&hrombocytopenia and hepatic

involvement may also manifest+incidence is about , I ")* 8eonatal lupus may appear up to @

wee7s after birth


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CONGENITAL HEART BLOCK

&he conse#uence of di6use myocarditisand


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MANAGEMENT DURING

PREGNANCY >rimarily of monitoring the clinical

conditions of maternal and fetal andmaternal laboratory values

&hrombocytopenia and proteinuriaresemble lupus disease activity

Lupus /are is confounded by theincrease in facial and palmar erythema

eighted for severity+ 1L20pregnancydisease activity index :1L2>D'!; andLupus activity index :L'!;


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1erial hematological studies maydetect changes in disease activity+hemolysis+ anemia+ reticulocytosis+

thrombocytopenia+ leu7openia 1erum transaminase activity re/ects

hepatic involvement+ as does arise in

serum bilirubin &he fetus should be closely observed

for adverse e6ects


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Fetal growth monitoring+ carefulattention is given to thedevelopment of hypertension

1creening for anti 110' and anti 110%antibodies+ and is found+ fetal cardiacfunction should be evaluated

'ntepartum fetal surveillance isdone+ including umbilical arteryvelocity


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PHARMACOLOGICAL TREATMENT

81'!D3 arthralgia and serositis aremanaged by 81'!D including'1>!9!8

&herapeutic doses should not beused after -@ wee7s

Low dose aspirin+ can be used safetythroughout pregnancy

!f L' andCor aAL antibodies positivetreated with aspirin ")) mg per day


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CORTICOSTEROID

Aorticosteroid3 severe disease ismanaged with corticosteroid+ such asprednison " to - mgC7g per day

'fter the disease is controlled+ this istapered to a daily dose of ") to ", mgeach morning

Aorticosteroid therapy can result in thedevelopment gestational or even type "diabetes

hen severe disease supervenes+usually with a lupus /are+ high doseglucocorticoid therapy is given


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9ecommendation pulse therapy consisting ofmethylprednisolon "))) mg per -@ hours for ?days+ than return to maintenance doses ifpossible

Flares were treated with prednison :)+, I "mgC7gCday and C or chloro#uine :-,) mgCday; >atient with '>1 previously complicated by

thrombotic events or fetal loss received aspirin:")) mgCday; and prednison " mgC7gCday :before"(B(; or unfractionated heparin thereafter

!n patient with positive anti 11'C9o or anti 11%antibodies no treatment was administered unlessthere were intrauterine cardiac complication+ inwhich case dexamethasone :@ mgCday; wasgiven


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IMMUNOSUPPRESSIVE

!mmunosuppressive and cytotoxic agentssuch as aEathioprine and cyclophosphamideare bene


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&he most widely used intravenous consistof monthly infusion with ,)) 0 "))) mgCm-for $ months

Followed by #uarterly infusion for "Bmonths

'dverse e6ects of treatment includenausea and vomiting+ alopecia+ bonemarrow depression and infection

>atient with class !J lupus nephritis treatedwith oral ,) I ")) mgCday for $ monthsfollowed by "B months aEathioprine "))mgCday


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ANTIMALARIA

'ntimalarials help control s7in disease

'lthough these agents cross the placenta+

hydroxychloro#uine has not beenassociated with congenital malformation

Flare were treated with prednisone :)+, I

" mgC7gCday; and C or chloro#uine :-,)mgCday;


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METHOTREXATE MTX

M&K is inhibitor that interferes with D8'synthesis

M&K for rheumatoid arthritis + other autoimmunedisease3 vasculitis+ myositis and 1L2

>roliferaive lupus nephritis resistance tocorticosteroid and intravenouscyclophosphamide were treated with wee7lydoses of .+, I ", mg

Ayclospporine , mgC7gCday is an inhibitor of t0cell proliferation+ usually is combined with M&K


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HORMONE

>rasteron :D2'; that was shown toameliorate nephritis

D2' can increase !L0- production by &0cell

>atient with 1L2 serum levels of D2' are

fe#uently decrased


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MONOCLONAL ANTIBODIES

'nti0cyto7ine monoclonal antibodies

'nti !L0") monoclonal antibodies3 levels of

!L0") are increased in patient with active1L2

!L0") levels correllate with disease activity

'nti0!L0$ monoclonal antibodies During active 1L2 high !L0$ levels are

found in serum


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CONCLUSION

" Lupus is heterogenous syndromes witha complex pathogenesis

- !ts genetic contribution and immune

system abnormalities includeoveractive % lymphocyte

? !nfections+ lupus /are+ end0organfailure and cardiovascular diseaseaccount for more death

@ inds of lupus3 lupus+ discoid lupuserythematosus+ subacute cutaneouslupus erythematosus+ drug inducedlupus and neonatal lupus


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, &he systems can be a6ected3 7idney+ lungs+central nervous system+ blood vessels+ bloodand heart

$ >redictions of pregnancy loss3 proteinuria+ '>1+thrombocytopenia hypertension

. >regnancy outcome is better if3 lupus activityhas been #uiescent at least $ months beforeconception

B Management3 primarily of monitoring theclinical condition of maternal+fetal andlaboratory value+ 81'!DC'spirin+ corticosteroid+immunosppressive+ cytotoxic agents+antimalaria and M&K


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3. Sulchan - Prof. Ssystemic Lupus Erythematosus in Pregnancy Revisi1

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