SYSTEMIC LUPUS ERITEMATOSUS

Definition Systemic lupus eritematosus (SLE) is a multisystem inflammation disease with unknown etiology. It maybe acute and fulminant or chronic remision and exaserbation that followed by various autoantibody. SLE is a multysistem autoimune disease that is characteri ed by appearen of a few abnormal immune reaction that result in many clinical manifestation. Epidemiology !revalence of SLE in each country various between "#$%&''.''' until (''%&''.'''. SLE often found in )igre# *hinese and philipine+s. familial tendention is also found. SLE can occur on all ages# but the most prevalence age is between &,-(' years old (reproduction periode). .he fre/uence on woman compare wiyh man is ,#, 0 $ 1&. 2n SLE induced by drugs the ratio is lower 31". Etiology and Pathogenesis Ethiology and pathogenesis of SLE is unknown. .here are many evidence that pathogenesis of SLE is multifactorial# including genetic# environment and hormonal factor againts autoimune response. 4enetic factor have the important role in vulnerability and disease expresion. &'"'5 SLE patient have familical history of SLE.the number of SLE on identic twins is higher than non-identic twins ("(-6$5 1 "-$5). .he recent studies shows that many genes that codes immune system factors are

participating. 7nother genes that including in this process is gene that code .-*ell receptors# imunoglobulin and citokin. )euroendocrine system is participated through the role againt imune system. 8oth neuroendokrine and immune system influenced each other. 7 study shows that prolactin could trigger imune response. .he hypothi e of SLE9s pathogenesis are1 there are one or few trigger factor to people with genetic predisposition that produce abnormal power againts .-cell and *:( that conse/uently the missing of .-cell tolerance againts self antigen that cause appear of autoreactive .-cell that induced the expansion of :-cell. 8oth of them produce autoantibody and memory cell. .he process is hypothesi e including sexual hormon# ;<light and many kinds of infection. 7utoantibody that produce in SLE wouldactivate complement that produce substance cause inflamation. .his inflamation support the symptom on target organ such as kidney# articulation# pleur# plexus choroideus# skin# etc. Clinical symptom .he history of disease are very various. It occur acut that followed by many disorder of the system on the body.it also can occur chronically attacked one system that progresively followed by imune system disorder. 2n chronic type there are remision and exaserbation. =emision might be occur by years. 2nset of this disease spontaneously or initiated by presipitation factor such as contact with ;<- light# infection of bactery and virus# drugs such as sulfa# abortion and phisical in>ury. Each onset usually

followed by general system such asfever# malaise# weakness# decrese appetite# decrese body weight and irritability. .he most fre/uent symptom is fever# sometimes followed by chilling. M sc los!letal 7rthritis and arthralgia are the most fre/uent symptom of SLE. It happen on interphalangeal# knee# elbow# and angkle. 8eside wheal and pain we can find articulayion effusion that usually includes class & ( noninflamation ) or class " (with inflamation). 7rtritis usually simetric without deformity# contractur or anxilosis. Sometimes rheumatoid nodule is found. 7vaskular necrosis can occur to a few places and mainly found on patien with high dose steroid treatment# where csput femoris is the most common places. M coc tan Skin# hair and mucous abnormality was found at ?,5 SLE. .he most fre/uent skin lesion is acute skin lesion# sub-acute skin lesion# diskoid# and livide reicularis. Spesific skin rush that usefull to make diagnose SLE is skin rush in form of butterfly (8utterfly rush). It perform erythema with a little edematous on nose and both of cheeks. @ith good treatment# this abnormality can trace better. 2n the body that exposure with ;<-light# it can occur skin rush because of photo-hypersensitivity. 7lopesia that occur can reduced if the disease remision completely. "idney .he most fre/uent manifestation is proteinuria and hematuria. Aypertension# nefrotic syndrom and renal failure is not often.only ", 5 of

SLE show abnormality of urine. .here are " kinds of pathology of abnormality renal difuuse lupus nefritis and membranous lupus nefritis. Ca#dio$as! la# 7bnormality of heart can be appear in form of mild pericarditis intill severe ( pericarditis effusion )# miokarditis iskhemic# and verrous endokarditis ( Libmann-Sacks) L ng Bild unilateral pleural effusion is often more than bilateral. %I t#ac! 7bdominal pain is found at ",5 of SLE# maybe followed by nausea and diarea. .he pain can be caused by steril peritonitis or arteritis of mesenterium small vessel and intestine that leading to intestinal ulcer. 7rteritis can also caused pancreatitis. Li$e# and spleen Aepatosplenomegali might be found but not often followed by icteric# it can be normal in a few months. Lymph glands Lymph gland enlargement is often found (,'5)# usually in form of diffuse limphadenopathy. &e#no s system !eripheral neuropaty is in form of sensoroc and motoric disorders usually temporary. *entral nervous system disorder consists of " main abnormality# organic psychosis and convulsion. !atient shows halutination% delution symptom of cerebral organic abnormality such as

disoriented# hard in counting# disability in recognation. *onvultion is occur in grandmall types. DIA%&OSIS .he criteria diagnosis based on 7=7 criteria. .here are && criteria to diagnosis SLE. If ( of && criteria is appear# the diagnosis can be established. .he criteria consists of1 &. rush on malar# in form of limited eritematosus# flat or elevated on malar# usually not reach the nasolabialis area ". discoid lesion# in form of eritematous pla/ue elevated with intact keratini ed with folikel obstruction. 3. photosensitivity# lesion caused by abnormal reaction againts sunlights. (. oral ulcer on oral or nasopharing usually not pain. ,. arthritis non erosive attack " peripheral articulation signed by pain# wheal or effusion. 6. serositis 1 a. b. pleuritis pericarditis

C. renal abnormality .a proteinuria# D '., g%day or 3E or .b cylinder cell# might be eritrosit# granuler hemoglobin# tubular or mixed. ?. neurological abnormality a. spontaneous convultion without trigger by drugs

b. spontaneous psychosis without trigger by drugs $. hematologic abnormality a. hemolitic anemia with reticulosis b. leucophenia# less than ('''% mm3at "x examination or more c. lymphopenia# less than &,''%mm3 at "x examination or more d. trombocitopenia# less than &''.'''%mm 3 withou trigger by drugs &'. immunology abnormality a. LE-cell or b. 7nti :)7# antibody againts native :)7 with abnormality titration. c. 7nti Sm1 antibody againts antigene of smooth muscles or d. False positive on serological test for syphilis. 7t least 6 months and strengthen by treponema pallidum mobilisation test or treponema antibody absorption fluoresence test. &&. 7nti )uclear 7ntibody

T'ERAPY A&D TREATME&T Co#ticoste#oid *orticosteroid is the most important in SLE treatment# it can use as topical for skin manifestation# with low dose for minor activity and high dose for mayor activity.

&SAIDs ;sually used for mild symptom# combine with corticosteroid to minimi e the effects of corticosteroid# its function as symptomatis treatment for artritis% atrhralgia# mialgia and fever. .he symptoms are eritema and oral ulseration. Imm nos p#esi$e d# gs ;sually combine with corticosteroids. 7pplication of this drugs based on the effects againts immune function# still debate until this time. It is only recommended for emergency case or diffuse lesion or membranous in renal that unresponse with high dose of corticosteroids# hypothesi ed function as anti viral. 2ther drugs are Betrotrexat# *yclosporin 7# intravenous Immunoglobulin# plasma infuse# =etinoid and the metabolit for diskoid skin lesion and sub-acute that refracter with ordinary treatment. :apson and .halidomide for severe skin lesion. T#eatment fo# complications .he most common complication is secondary infection. In cardiopulmonar it can occur heart failure caused by miokarditis# pleural effusion and pericarditis effusion. =enal abnormality could occur in form of mild renal faulire until severe renal failure. In this condition consider for clinical giving the diuretics# anti hypertension drugs# dialisis and renal transplantation. 7nticoagulantia and anticonvulsion drugs can be considered.

PRO%&OSIS .he using of corticosteroid drugs has shown good remision. =egularity and planning in treatment with corticosteroids# using of antibiotics# apply of immune supresive drugs# anti hypertension# dialisis and renal transplantation# it decreased , years survival rete from ,'5$,5.

CASE REPORTS 7 "$ years old girl was admited to internal medicine departement of 4eneral Aospital :=. 7hmad Buchtar 8ukittinggi on February "# "''3 with1 *hoef complain1 8reathless since " months ago P#esent illness histo#y1 8reathless since " months ago# increased since C day before admitted. 8reathless not influenced by wheather# activity# emotion# position and food. 7ppear of erithematous spot all over the body since " months ago# it beginning with erithematous spots and swollen on the face#hands and foot which is no itch . .he patient got high fever since " months ago# which is uncontinuing# not chilling and not sweating. @eakness of the body since " months ago. 7rticulation pain felt since &#, months ago *ough since &#, month ago# sometimes productive.

7ppetite decrease since illness. )ause and vomit is negative. Bixturation and defecation were normal

P#e$io s illness histo#y( ) )othing is important *amilial Illness 'isto#y( )one of the family members had disease like this.

Physical E+amination ( ,ital Sign( - 4eneral appearance - level of consciousness - blood pressure - pulse rate - respiratory - temperature S!in( - cyanotsis - pale 1 (-) 1 (-) 1 moderate illness 1 composmentis cooperative 1 &''%C' mmAg 1 $6 x%menit 1 3" x%menit 1 3?#3 '*

- erithematous spot all over the whole body. hyperpigmentation on the face (E) butterfly rash on the face (E)

Lymph node 1 no enlargement 'ead( ) .hinning of the hair

&ec!( -

Eye1 con>ungtiva not anemic# sclera not icteric Ears# nose and mouth1 was dry

G<!

1 , - " cmA"2

)o enlargement of lymph nodes and thyroid gland

Chest ( Shape1 )ormochest

L ngs( Inspection 1 symetric movement# thoracoabdominal breathness# fre/uency 3" x%minute !alpation 1 fremitus were decreased on both lung from the <III inter costal space !ercussion 1 sonor# deafness on both lung from the <III inter costal space 7uscultation 1 vesiculer from the <III inter costal space# ronchi (-)# whee ing (-) 'ea#t( Inspection !alpation 1 ictus was not visible 1 ictus was palpable on the left midclavicula line of the < Inter *ostal Space# with no strong pulsation and & fingers wide. !ercussion 1 left border was on the left midclavicula line of the < Inter *ostal Space. =ight border was LS:.

;pper border was Inter *ostal Space II. 7uscultation 1 irreguller rhythm# B&DB"# !"H7"# sistolic and diastolic murmur(-). A-domen( Inspection !alpation !ercussion 7uscultation .ac!( Inspection was simetric# no decubitus# and no pressure pain and hammer pain. E+t#imity( - physiological reflex - patologycal reflex - swollen extrimity - tremor - sianotic fingers La-o#ato#y *inding /L*0 .lood ( 1 $#? gr5 1 3.(''%mm3 1 3,%Aour 1 '%&%"%,'%((%( 1 3#3 million 1 (-) 1 (-) 1 (-) 1 E%E normal 1 -%- normal 1 distended abdomen (-) 1 liver not palpable# Spleen not palpable# ballotement (-) 1 tympani 1 peristaltic sound was (E) normal

) Ab - Leucocyte - 8S= - :iff *ount - Erytrocyte

- Aematocrite - =eticulocyte - .rombocyte - 7nemia - .otal protein - 7lbumin - 4lobulin

1 "C vol5 1 "#"5 1 &(C.'''%mm3 1 )ormositic )ormokrom 1 ,#&& g%dl 1 "#$' g%dl 1 "#"& g%dl

- .otal bilirubin 1 '#66 mg%dl - 8il. :irek - 8il. Indirek - ;reum - Ireatinin - S42. - S4!. - Abs7g - 7nti A8s 1 '#&( mg%dl 1 '#," mg%dl 1 &$6 mg%dl 1 '#? mg%dl 1 $? ;%I 1 "$ ;%I 1 (-) 1 (-)

- 7lkali Fosfatase 1 6CC ;%I 1o#!ing Diagnosis( Systemic Lupus Erythematosus Diffe#ential Diagnosis ( :rug Eruption The#apy( 8edrest. Low salt II and Aeart II diet

I<F: : ,5 &' hour % kolf Lasic &x & tab I*l 3 x & :igoxin " x & tab

Planning e+amination1 blood# urine and feces rutine examination rontgen thorax# E*4 Lipid profile# ureum and kreatinin serum S42.# S4!. *omplete blood investigation ()a# I# 7lb# 4lob ) 7F8 sputum