Case Report Kepada Yth.

Non-Infection Unit
LUPUS NEPHRITIS DUE TO SYSTEMIC LUPUS ERYTHEMATOSUS
Presenter : Rani Safitri
Day/Date :
Supervisor : dr. Rosmayanti, Sp. A (K)
Introduction
Systemic lupus erythematosus (S!, or lupus), a rheumatic disease of un"no#n cause, is
characteri$ed %y autoanti%odies directed a&ainst self anti&ens.
'
(any factors, includin& &enetic
predisposition, ethnic ori&in, hormones, environmental factors, and medications potentially tri&&er
immune dysre&ulation.
',)
upus most commonly affects #omen of child%earin& a&e ()*+,* years), at a
female to male ration of -:' to '.:'.
),/
Asians #ith S! had hi&her rates of renal involvement than
#hites did, and cardiovascular involvement #as a leadin& cause of death in Asians.
,

Systemic lupus erythematosus is a prototypic autoimmune disease characteri$ed %y
autoanti%ody production and immune comple0 formation or deposition in tar&et or&ans such as the
"idney..
.
1he etiopatho&enesis involves a%normal anti%odies and polyclonal 2 cells3 ho#ever the
disease etiolo&y is not completely understood. 4enetic propensity %ecause of human leu"ocyte
anti&ens, se0 hormones, environmental e0posure such as sun, infection, and certain dru&s, such as sulfa
anti%iotics, are considered predisposin& factors. 1he disease is characteri$ed %y disordered immunity
involvin& auto+reactive 2 and 1 lymphocytes. Auto+anti%odies form immune comple0es #ith tissue
anti&ens. 1hese immune comple0es accumulate in small vessels of or&ans #here they stimulate local
inflammation %y activation ofcomplement path#ays and cause consumption of complement.
5

6ypocomplementemia (7/ and 7, deficiency) is found in three 8uarters of S! patients and if
present may help #ith a lupus nephritis dia&nosis. (ast cell de&ranulation and local infiltration of
macropha&es and neutrophils causes further tissue dama&e. Additionally, there is an ina%ility of 1+cells
to suppress the 2+cell clones %ecause of1+cell dysre&ulation. !0cess 7D,91+cell activity and deficient
7D:9 cytoto0ic/suppressor function are present. Another immune system defect in S! involves
a%normal control of apoptosis and defective clearance of cellular de%ris creatin& cellular anti&en
e0posure and incitin& further immune response. upus nephritis is an immune comple0 mediated
disease that results from immune system disorder and dysre&ulation resultin& in #idespread
inflammation and immune+comple0 induced "idney dama&e.
5

1he clinical manifestations varies, dependin& on the or&an involved. 1he most common
cutaneous manifestations is malar or %utterfly #hich may %e photosensitive.
'
(usculos"eletal findin&s
include arthral&ia, arthritis, tendinitis, and myositis. Serositis can affect pleural, pericardial, and
peritoneal surfaces. (any patients #ith lupus e0perience memory loss or other co&nitive dysfunction in
their disease course. Renal disease is manifest %y hypertension, peripheral edema, retinal vascular
chan&es, and other clinical manifestations associated #ith electrolyte a%normalities, nephrosis, or acute
renal failure.
'
Dia&nosis is %ased on classification criteria esta%lished %y the American 7olle&e of
Rheumatolo&y (A7R). A minimum of , of the '' A7R criteria should %e met in order to 8ualify as
S!. 1he '' A7R criteria are %ro"en into systems: cutaneous (malar rash, discoid rash,
photosensitivity), oral ulcers, musculos"etetal nonerosive arthritis, serositis (cardiopulmonary pleuritis
or pericarditis), renal, neurolo&ical disorder #ith sei$ures or psychosis due to un"no#n causes, and
la%oratory (haematolo&ic, immunolo&ic, and antinuclear ati%ody).
)
1reatment modalities include non pharmalo&ical approach, steroids, antimalarials, and
cytoto0ic/immunosuppressive a&ents.
)
7orticosteroids control symptoms and autoanti%ody production
in lupus. 1reatment #ith corticosteroids has improved "idney disease and the rate of survival. Patients
#ith systemic disease are often started on ';) m&/"&/), hr of oral prednisone in divided daily doses.
<hen complement levels increase to #ithin the normal ran&e, the dose is carefully tapered to the
lo#est effective dose. (ethotre0ate, cyclosporine, and mycophenolate mofetil are used as steroid+
sparin& a&ents.=
The aim of this paper is to report a case of systemic lupus erythematosus in a 14 years old girl.
Cae
>ame : <?P
A&e : '. years
Se0 : @emale
Date of Admission : (ei ')
rd,
,)*',
<?P, female, '. years old, ?ndonesian, admitted to 6. Adam (ali" 4eneral 6ospital on (ay ')
rd
,
)*',, #ith main complaint of pale she has %een e0periencin& since ' month a&o. 6istory of spontan
%leedin& (9) and also feces #ith %lac" colour (9), nose%leedin&(9), &ums %leedin& (9), red spot on the
s"in (9), malar rash (9) has %een e0periencin& for ' month a&o and if patient e0pose to the sun li&ht the
lession &ettin& #orse. 7ou&h (9) since ) #ee"s a&o, sputum (+) no history direct contact #ith adult
person #ith cou&h, common cold (+), nausea (+). @ever (9) e0periencin& %y the patient since ' month,
characteristic of fever #as su%fe%ris and %ac" to the normal #ith antipiureti", sei$ure (+) shiver (+).
(alaise (9) since ' month, hairfall has %een e0perience since 5 month a&o, history of %ac"%one pain
(9) especially #hen inspiration. o#er appetite (9), the hi&hest %ody #ei&ht she reached #as ,)"&
around '* month a&o, no# she #ei&ht /. "&. history replace of thrush (9) since ' month a&o. Patient
has %een e0periencin& amenorrhea for the past : months. 1he history of menstruation #as found not
re&ular and she had menarchy at a&e of thirteen. Previously, the patient #as admitted for ' #ee" at
1e%in& 1in&&i 6ospital #ith dia&nose anemia ec (A), she had under&one %lood transfusion as much ,
%a& PR7. Bn ')
th
(ay )*',, the patient #as dischar&ed from 1e%in& 1in&&i 6ospital and #as reffered
to 6.Adam (ali" 4eneral 6ospital. She #as admitted here for t#o #ee" from ')
th
may to ):
th
may
#ith the dia&nosis of Severe S!.
Hitor! o" pre#iou i$$ne : Denied %y patientCs mother
Hitor! o" pre#iou %edication : >e&ative
Hitor! o" $a&or : >ormal delivery, handled %y mid#ife, cried
as soon as %a%y #as %orn, no cyanosis the
patient is 'st child.
Hitor! o" 'ro(th and
De#e$op%ent : 4ro#th and development #hen toddler
suita%le #ith toddler in same a&e. 6e #as :
th

&rader elemantary school student.
Hitor! o" "eedin' : * ; 5 months : %reast feedin&
5 ; ') months : porrid&e mil", %reast feedin&
' years ; no# : re&ular diet
Hitor! o" i%%uni)ation : 7omplete immuni$ation
Ph!ica$ e*a%ination
Preen tatu
Sensorium : 7ompos mentis
1emperature : /5,.D7
6eart Rate : '), %pm, re&ular, murmur (+)
Respiratory Rate : ): 0/minute, re&ular, rales (+)
2lood Pressure : ''*/E* mm6& normal
<ei&ht : /. "& 22/F G
6ei&ht : '.. cm 12/F G
>utrition Status : 22/12 G
Dyspnea, cyanosis, icteric, and edema #ere not present
Loca$i)ed Statu
S"in (alar rash (9)
6ead 6air #as easily pulled off (9)
@ace (oon face (9), 2utterfly rash (9), Acne at %oth chee"s (+)
!yes i&ht refle0: (9/9), pupils #ere isochoric, no pale of conHunctiva palpe%ra
inferior
>ose >ormal in appearance
!ar >ormal in appearance
(outh 6yperemic (+)
1oun&e/ 1eeth >ormal in appearance
1onsil/ Pharyn0 >ormal in appearance
>ec" !nlar&ement of lymph nodes (+)
2ac" (iliary spots (+)
1hora0 Symmetrical fusiform, no retraction,
6R: '), %pm, re&ular, murmur (+)
RR: ): rpm, re&ular, rales (+/+)
A%domen Soepel, peristaltic (9) normal, liver/spleen: not palpa%le
Pelvic 7osto+verte%ral an&le tenderness (+)
!0tremities Pulse -'), %pm, re&ular, p/v #as ade8uate, #arm e0tremities, 7R1 I)J
Striae on "nee (9)
2lood pressure ''*/E* mm6& (>: '*5+')*/ 5/+EE)
Pu%erty sta&e 1anner sta&e 2/P/
La&orator! "indin' on Ma! +,
rd
,-+.:
Co%p$ete /$ood
Count
He%ato$o'! Unit Reu$t Re"erence
He%o'$o&in 0H1/2 &K -.'* ').*+',.,
Er!throc!te 0R/C2 '*
5
/mm
/
).,* ,.)*+,.:E
Leucoc!te 03/C2 '*
/
/mm
/
'*./* ,..+''.*
He%atocrite K )../* /:+,,
Thro%&oc!te 0PLT2 '*
/
/mm
/
'E' '.*+,.*
MC4 @l '*..,* :.+-.
MCH p& /E.-* ):+/)
MCHC &K /5.** //+/.
RD3 K )).)* ''.5+',.:
MP4 @l -..* E.*+'*.)
PCT K *.'5
PD3 @l ''.,
Di""te$ Count
Neutrophi$ K EE./* /E+:*
L!%phoc!te K ''.E* )*+,*
Monoc!te K :.** )+:
Eoinophi$ K ).E* '+5
/aophi$ K *./** *+'
Neutrophi$ A&o$ute '*
/
/L E.-E ).E+5..
L!%phoc!te A&o$ute '*
/
/L '.)* '..+/.E
Monoc!te A&o$ute '*
/
/L *.:) *.)+*.,
Eoinophi$ A&o$ute '*
/
/L *.): *+*.'
/aophi$ A&o$ute '*
/
/L *.*/ *+*.'
Li#er
A$&u%in &/d /.) + ,..
Car&oh!drate
Meta&o$i%
Rando% /$ood 1$ucoe m&/d '/).-* I)**
Rena$
Ureu% m&/d 'E.E* I.*
Creatinin m&/d *./. *,.E+*,:E
E$ectro$!te
Natriu% m!8/d '/) '/. ; '..
Ka$iu% m!8/d /., /,5 ; .,.
C$oride m!8/d '*, -5 ; '*5
3or5in' Dia'noi6 Severe systemic lupus erythematosus
Mana'e%ent 6
(ethylprednisolone /+/+/
Diet common food '::* "cal #ith E: &ram of protein
P$annin' 6
+ 2alance fluid/ 5 hour
+ Dipstic" urine at mornin&
+ 7onsult to endocrinolo&y
+ 7hec" complete %lood count, R@1, urinalysis, electrolyte and %lood &lucose level.
Dicuion
A '.+year+old ?ndonesian &irl presented #ith features of S! at the a&e of '.. 6er initial
clinical manifestations #ere prolon&ed erythematous rash over the malar re&ion. She also have noticed
that her erythemstous rash &et #orse due to sunli&ht esposure and thinnin& of hair. She havin& a sudden
and continous pain at the lo#er %ac" till it limits her daily activities. 1he pain on her lo#er %ac" ma"es
her difficult to inspiration. She is also havin& amenorrhea since : months a&o. 1he patient first had a
menarche at the a&e of '/ and her history of menstruation #as normal. 6er current pu%ertal sta&e is 2/,
P/. 6er %lood pressure #as #ithin normal limits at ''*/E* mm 6&.
a%oratory findin&s included hemo&lo%in of -.'*&K, #hite %lood cell count of '*./*K (EE./*
neutrophils, ''.E* lymphocytes, and :.** monocytes), and platelet count of 'E',***. 1he patient is
under&oin& severe S! protocol treatment #ith methylprednisolone.
An estimated '* to )*K of patients e0perience the onset of S! prior to adulthood. (ore
precise estimates are difficult due to a lac" of a clear a&e limit for the dia&nosis of pediatric S!. 1he
ma0imum a&e at dia&nosis most commonly used to define pediatric S! is '5 years %ut a&es ran&e
from ', to )* years in various studies.=M ?n this case, this patient first dia&nosed as S! at the a&e of '..
7utaneous symptoms include malar rash (%utterfly) or discoid rash3 additionally, scarrin&
alopecia (chronic hair loss), and mucosal ulcerations are fre8uent in childhood S!. 1he malar rash
occurs in one third to one half of children at the onset ofdisease and is considered hi&hly su&&estive of
S!. 1he rash is usually raised and #ell demarcated #ith sparin& ofthe nasola%ial folds.
(aculopapular rashes in S! can occur any#here on the %ody as a manifestation of vasculitis. 1hey
fre8uently occur in sun e0posed areas of the face and upper chest.
E
Pediatric S! often presents #ith more acute and severe disease features than aS! %ased on
studies providin& direct comparisons. Almost all pu%lished research su&&ests a hi&her fre8uency of
renal, neurolo&ical, and hematolo&ical involvement #ith pediatric S! than #ith a S! at the time of
dia&nosis.?n a 7anadian inception cohort of 5E pediatric S! patients the avera&e disease activity
score, as measured %y the S! Disease Activity ?nde0 (S!DA?), #as '5.: at dia&nosis %ut only -./ in
the comparison &roup of '/' patients #ith aS! (p G *.***'). 1he most pronounced differences in
disease activity %et#een aS! and pediatric S! pertain to the renal or neurolo&ical or&an systems.
E
<hen comparin& pre+pu%ertal to post+pu%ertal onset of pediatric S!, the former &roup
presents more often #ith hemolytic anemia and renal involvement #hereas in the latter &roup
cutaneous and musculos"eletal features are more common at disease onset. A%out one third of the
children and adolescents #ith S! present #ith anemia, throm%ocytopenia, or lymphopenia at the time
of S! onset.
E
1raditional ris" factors for osteoporosis that contri%ute to impaired %one health in aS! are
unli"ely important in pediatric S!. Possi%ly due to inflammation, corticosteroid use, reduced physical
activity, inade8uate sun e0posure, and lo# calcium and vitamin D inta"e, children and adolescents #ith
S! are often una%le to reach their pea" %one mass. @or reasons not fully understood, there is a hi&her
prevalence of vitamin D deficiency in children and adults #ith S! as compared to the &eneral
population.
E
Depression is the most common mood disorder in children and adults #ithout apparent
differences in prevalence %et#een &roups. 1he 4rupo atino Americano de !studio del upus
(4AD!) cohort reported a si&nificantly hi&her prevalence of pseudotumor cere%ri, transient
ischemic attac", and sei$ures in their pediatric S! cohort #hen directly compared #ith aS!. ?n this
case, this patient #as had a previous history of sei$ure and e0hi%its the neurolo&ic manifestation of
S!.
E
(enarche in females #ith pediatric S! is, on avera&e, delayed %y one year. 1he delay in
pu%erty pro&ression increases #ith lon&er disease durations and the hi&her cumulative doses of
corticosteroids used for treatment (R)N *./3 pI *.**-). 1ransient or permanent amenorrhea has %een
reported in ')K of )-: adolescents #ith pediatric S! and is positively correlated #ith %oth the
presence of disease activity and dama&e.
E
Dia&nosis is %ased on classification criteria esta%lished %y the American 7olle&e of
Rheumatolo&y (A7R). A minimum of , of the '' A7R criteria should %e met in order to 8ualify as
S!.
)
!levated A>A titers are often present in children #ith active lupus. 1his is an e0cellent screenin&
tool, althou&h A>A can %e found #ithout any disease or can %e associated #ith rheumatic, and other
conditions, in either #ay, is far from specific.
'+=M
1he antinuclear anti%odies (A>A) test is hi&hly
sensitive #ith a positive result in N-.K of S! patients.
'
Si&nificant titres are accepted to %e of '::* or
&reater.
:
Steroids, cyclophosphamide, a$athioprine, and mycophenolatemofetil remain first+line
therapeutics for treatment of >. 1hese nonselective immunosuppressants have much improved the
response rates of acute manifestations and the overall mortality of S!3 ho#ever, the lon&+term
outcomes of > have not further improved durin& the last /* years. 6i&h+dose steroids and
cyclophosphamide are fre8uently associated #ith severe side effects, and infections contri%ute to the
overall mortality in S!.
-
Patients #ith S! of all a&es older than 5 years are e8ually li"ely to %e prescri%ed antimalarials.
Adults and children #ith S! are compara%le in their use of non+steroidal anti+inflammatory
medications, e0cept for cycloo0y&enase ) inhi%itors #hich appear to %e less commonly prescri%ed to
children and adolescents.?n children #ith severe >, cyclophosphamide use is associated #ith %etter
renal survival compared to corticosteroids therapy alone.
E
Su%%ar!
1his paper reports a case of a '. years old female dia&nosed #ith systemic lupus erythematosus.
Dia&nosis is made %ased on the dia&nostic criteria %y American 7olle&e of Rheumatolo&y (A7R). 1his
pasien meets 5 of '' criteria consistin& of dermatolo&ic manifestation such as the classic symptom of
S!, malar rash, photosensitivity, follicular plu&&in&, musculos"eletal manifestation such as
involvement of spine osteopenia. 1his patient is treated #ith hi&h+dose of intravenous
methylprednisolone for suppressin& the autoimmunity #hich %ecame the mainstay of therapy for
children #ith severe S!. 1he patient &ot oral therapy #ith methylprednisolone is started at a dose of
,m&. As many as ).K of patients may e0perience remissions, sometimes for a fe# yeras, %ut these are
rarely permanent. 1he leadin& causes of death in the first decade of disease are systemic disease
activity, renal failure and infections.