Case Report

Kepada Yth.

Non-Infection Unit
SYSTEMIC LUPUS ERYTHEMATOSUS
Presenter

: Suwenny (100100059)
Cindy (100100063)

Day/Date

: Tuesday/ April 1st 2014

Supervisor in charge : dr. Oke Rina Ramayani, M.Ked(Ped), Sp.A

Supervisor

: dr. Fera Wahyuni, M.Ked(Ped), Sp.A

Introduction
Systemic lupus erythematosus (SLE, or lupus), a rheumatic disease of unknown cause, is
characterized by autoantibodies directed against self antigens. 1 Many factors, including genetic
predisposition, ethnic origin, hormones, environmental factors, and medications potentially
trigger immune dysregulation. 1,2 Lupus most commonly affects women of childbearing age (2040 years), at a female to male ration of 9:1 to 15:1. 2,3 Asians with SLE had higher rates of renal
involvement than whites did, and cardiovascular involvement was a leading cause of death in
Asians.4
The development of SLE is a complex immune process that is brought about by
dysregulation of B- and T-lymphocytes, the production of auto-antibodies, and the formation of
immune complexes. While it is known that the immune system plays a role in the development
of the disease, what causes the immune system to function abnormally is unknown. 2 Children
with lupus present with diverse and often severe manifestations. Children most frequently
present with fever, fatigue, hematologic abnormalities, arthralgia or arthritis, rash, and renal
disease. Symptoms may be intermittent or persistent.1
The clinical manifestations varies, depending on the organ involved. The most common
cutaneous manifestations is malar or butterfly which may be photosensitive. 1 Musculoskeletal
findings include arthralgia, arthritis, tendinitis, and myositis. Serositis can affect pleural,
pericardial, and peritoneal surfaces. Many patients with lupus experience memory loss or other
cognitive dysfunction in their disease course. Renal disease is manifest by hypertension,

peripheral edema, retinal vascular changes, and other clinical manifestations associated with
electrolyte abnormalities, nephrosis, or acute renal failure. 1
Diagnosis is based on classification criteria established by the American College of
Rheumatology (ACR). A minimum of 4 of the 11 ACR criteria should be met in order to qualify
as SLE. The 11 ACR criteria are broken into systems: cutaneous (malar rash, discoid rash,
photosensitivit), oral ulcers, musculosketetal nonerosive arthritis, serositis (cardiopulmonary
pleuritis or pericarditis), renal, neurological disorder with seizures or psychosis due to unknown
causes, and laboratory (haematologic, immunologic, and antinuclear atibody).2
Treatment modalities include non pharmalogical approach, steroids, antimalarials, and
cytotoxic/immunosuppressive agents.2 Corticosteroids control symptoms and autoantibody
production in lupus. Treatment with corticosteroids has improved kidney disease and the rate of
survival. Patients with systemic disease are often started on 12 mg/kg/24 hr of oral prednisone
in divided daily doses. When complement levels increase to within the normal range, the dose is
carefully tapered to the lowest effective dose. Methotrexate, cyclosporine, and mycophenolate
mofetil are used as steroid-sparing agents.1

The aim of this paper is to report a case of systemic lupus erythematosus in a 14 years old girl.
Case
SW, female, 14 years old, Indonesian, admitted to H. Adam Malik General Hospital on March
10th, 2014, with main complaint of joints pain she has been experiencing for the last three
months. The pain was first started on joints of lower extremities and then followed by the joints
of upper extremities. Swelling and weakness was found on lower extremities in the past two
months and it got worse. Decreased appetite and body weight happened in the past two months.
The highest body weight she ever reached was 60 kg, now she weighs 47 kg. She admitted pale
since 3 months ago and that she has undergone transfusion of 4 red-coloured blood bag at Haji
Hospital. One week ago, her stool was black and her nose bleed. History of dispnoe was positive
in the past two months, not related with activity and weather. Cough was also positive, with
sputum but no blood in the past two months. Urine was red-colored this past few days.

Before she was admitted to Adam Malik, she was a patient at Haji General Hospital for about 2
weeks. A chest x-ray was done there and the result showed CTR >50.5, consolidation on the
bottom of the left lung, with the impression of she having pneumonia & left pleural effusion. A
reference letter from Haji Hospital stating the suspected diagnosis she had was hemolytic
anemia, Scroplodema TB, and cardiomegaly.
Physical examination
Consciousness: Compos Mentis. Body weight : 47 kg, Body Height : 160 cm.
Dyspnea, cyanosis, icteric, anemic, and oedema were not present
Temperature was 37,0C
Head

Hair was easily pulled off (+), spacious (+), Malar rash (-)
Eyes: light reflex: (+/+), pupils were isochoric, no pale of conjunctiva

Neck

palpebra inferior; Ear/nose/mouth: normal in appearance

Enlargement of lymph nodes (-)

Thorax

Symmetrical fusiform, no retraction

HR: 104 bpm, regular, murmur (-)

Abdomen

RR: 28 rpm, regular, rales (-/-)

Soepel, peristaltic (+) normal, liver/spleen: not palpable
Abdominal pain (+) in the past two months

Extremitie

Pulse 104bpm, regular, p/v was adequate, warm extremities, CRT <3

Swelling on ankle joint sinistra et dextra (+/+).

ROM, flexion and extension limited, pain (+/+).
Blood pressure 100/70 mmHg (N: 106-120/ 63-77)

Laboratory finding on March 10th 2014:

Hemoglobin 12.4 gr/dL, Hematocrite 36.6%, Leucocytes 5.480/mm3, Platelet 127.000/mm3
MCV 86.7 fL, MCH 29.4 pg, MCHC 33.9 g%, RDW 19.9%, Erythrocyte 4.22x106/mm3
Neutrophils 87.6%, Lymphocytes 5.8%, Albumin 2.3 g/dL
Ureum 28.8 mg/dL, Creatinine 0.44 mg/dL, Uric acid 15.3 mg/dL
Working Diagnosis: suspected SLE

Management :

Prednisone 1-2 mg/body weight/day = 47-94 mg/day 50 mg/day 4-4-3

Ibuprofen 2 x 500 mg
Diet common food 2040 kkal + 94 gr protein

Planning :
-

Consult to immunology, respirology, cardiology, & neurology division

Check ANA Test, ds-DNA, C3, C4

Follow up on March 10th March 18th 2014

S : fluctuating fever (+), joint pain (+), cough (+), decreased conciousness (+), restlessness,
lesion on lips (+), GCS 12 (E4 V2 M6), seizure (+/-)
O: Sens: CM, Temp: 37.6-39.2 oC. BW : 47 kg.
Head

Eyes: light reflex: +/+, isocoric pupil, pale of conjunctiva palpebra inferior (-),

Neck

Ear/nose/mouth: normal in appearance, butterfly rash (-)

Enlargement of lymph nodes (-)

Thorax

Simetrical fusiform, epigastrial retraction

HR: 158 bpm, regular, murmur (-)

Abdomen

RR: 30 rpm, regular, ronchi (-/-)

Soepel, peristaltic (+) N, impression of ascites

Extremities Pulse 158 bpm, regular, pressure and volume were adequate, BP 110/80 mmHg,
Swelling on ankle joint sinistra et dextra (+/+)
A : suspected SLE, seizure ec lupus cerebral + Hematuria ec UTI or Acute Glomerulonephritis
P:
Regular food diet 2040 kkal + 94 gr protein changed to SV diet on 11th
Ibuprofen 2 x 500 mg (10-11th)
Prednisone 4-4-3; 50 mg/day (started on 10th)
IVFD D5% NaCl 0.45% 80 gtt/i micro (started on 11th) IVFD D5% NaCl 0.45% +

Calcium gluconate 20 cc + KCl 25 mEq 40gtt/i micro (14th-18th)

Strict follow-up, monitor for repeated seizure
Inj. Phenobarbital LD 800 mg IV in 20 cc D5% in 20 minutes continued by MD 100

mg/12 hours (started on 11th)

Inj. Phenytoin 10 mg/body weight/day 400 mg/day (started on 11th)
Inj. Novalgin 500 mg/ 8 hours (started on 11th)
Inj. Ceftriaxone 1 gr/12 hours (started on 11th)
Correction of albumin 20% 207 cc (13th & 18th)
Dipstick : Uro 68+/ Bil 103+++/ Blood (+++)/ Prot (++++)/ Specific gravity (1.025)/ pH 6

Laboratory finding on March 11-15th 2014:

Bilirubin total 2.01 mg/dL, Bilirubin direct 1.85 mg/dL, ALP 308 U/L, AST 107 U/L, ALT 16
U/L, Albumin 1.9 g/dL, LDH 952 U/l
ANA Test 5.40 (weak), Anti ds-DNA 50.7 (weak), AFP 1.13 ng/ml, blood -HCG O mIU/ml
Bicarbonate (HCO3) 17.1 mmol/l, BE -6.7 mmol/l
Electrolyte Na 132 mEq/l, K 2.7 mEq/l, Ca 7.5 mg/dL, P 2.1 mEq/l
Urinalysis Dark Yellow, Bil (+), Ket (+), Pro (++), Uro (+), Nit (+), Blo (+)
Urine Sediment Erythrocyte 30-40, Leucocyte 20-30
Ureum 34.4 mg/dL, Creatinine 0.61 mg/dL, Uric acid 11.6 mg/dL
PT 18.6 (N 12.7), INR 1.52, aPTT 31.9 (N 33.2), TT 21 (N 17.3)
Consultation results from immunology division on March 11th 2014:
Advice : Check ANA, Anti dsDNA, LED, CRP, complement C3 & C4
Consultation results from neurology division on March 11th 2014:
Diagnosis : SLE
Advice : Head CT Scan
Consultation results from respirology division on March 13th 2014:
Advice : Mantoux test, gastric lavage, hypoalbuminemia correction
Consultation results from cardiology division on March 14th 2014:
Echocardiography fair LV function + mild MR + moderate segmented PE
Advice : Check for ASTO, CRP, throat swab & ECG, furosemide 2 x 40 mg + spironolactone 2 x
25 mg, repeat echocardiography on 27th
Head CT Scan Results on March 15th 2014:
Sulcus and gyri were good, ventricle & cysterna system were good, differentiation of white and
gray matter were good, no pathological hipohiperdense lesion, movement of middle line were
not seen, both of bulbous oculi were intact, bones & soft tissues were good
Conclusion : No anomaly found at the moment

Consultation results from psychiatry department on March 16th 2014:

Diagnosis : lupus nephritis
Advice : urine culture & renal USG
Laboratory findings on March 17th 2014:
ASTO <200, HbsAg (-), Anti HBs (-), Anti HCV (-), ANA Test 112 (strong positive), Anti dsDNA 692 (moderate positive), qualitative CRP (-)
Renal & Urinary Tract Ultrasonography Results on March 18th 2014:
Both renal were in normal size, neither stone nor widening of pelvicalyx system were seen.
Bladder was not full, collection of fluid was seen in the sorrounding,
Conclusion : No anomaly on renal was seen, Ascites
Consultation results from psychiatry division on March 18th 2014:
Diagnosis : organic mental disorder
Advice : Haloperidol 1,5 mg 2 x tab
Follow up on March 19th 29th 2014
S : fever (-/+), diarrhea (+) on 19-20th , seizure (-), GCS 12 (E4 V2 M6), brown-coloured urine
(19th), reddish urine (20th), hairfall (25th)
O: Sens: CM, Temp: 36.8-37.0 oC. BW : 47 kg.
Head

Eyes: light reflex: +/+, isocoric pupil, pale of conjunctiva palpebra inferior (-),

Neck

Ear/nose/mouth: normal in appearance, butterfly rash (-), discoid rash (-)

Enlargement of lymph nodes (-)

Thorax

Simetrical fusiform, retraction (-)

HR: 90 bpm, regular, murmur (-)

Abdomen

RR: 24 rpm, regular, ronchi (-/-)

Soepel, peristaltic (+) N, ascites (+) minimal, Hepar: palpable at 5cm below

costae arch. (19th) Spleen : not palpable

Extremities Pulse 90 bpm, regular, p/v was adequate, BP 90/60 mmHg, CRT<3, pretibial
edema (+/+)
A : SLE + lupus nephritis + Mild MR + Moderate segmented PE + organic mental disorder +
hepatitis + seizure ec (?)

P:

MII diet 2000 kkal + 100 gram of protein (started on 19th)

IVFD D5% NaCl 0.45% 60 gtt/i (micro)
Inj. Ceftriaxone 1 gr/12 hours/IV changed to Inj. Meropenem 1 gr/12 hours/IV (on 22th)
Phenobarbital Maintenance Dose tapp-off to 85 mg in 200 ml D5% in 20 minutes (19th)

then changed to oral phenobarbital 2 x 60 mg (23rd) 2x 50 mg (25th)

Inj. Phenytoin Maintenance Dose tapp-off to 85 mg in 200 cc D5% in 20 minutes (19th)

then changed to oral phenytoin 2 x 60 mg (23rd) 2 x 50 mg (25th)

Inj. Methyl Prednisolone 30 mg/kg/day (max 1 g/day) in 100 ml D5% for 1 hour 100
gtt/i micro for 3 consecutive days changed to 4 mg oral methyl prednisolone 0.5-2

mg/kg/day (max 1 g/day) 4-4-4

Furosemide 2 x 1 tab (40 mg) (started on 19th)
Spironolactone 2 x 25 mg (started on 19th)
Urdafalk 2 x 250 mg (started on 19th)
Halloperidol 1.5 mg 2 x tab (started on 19th) tapp-off to 1 x tab (on 25th)
Zinc 1 x 20 mg (started on 21st)
Hydroxychloroquin 2 x 150 mg (started on 22th)
Bolus 24 cc of Calcium Gluconate in 24 cc of D5% in 20 minutes (23th)
IVFD 2a 85gtt/i for 24 hours (23th)
CPA 500-1000 mg/ml with Mesna 60% (23th)
Calnix 2 x 1 tab (started on 26th)
Dipstick (21st) : Leu 70+/ Nit (-)/ Uro 0.2 (3.5)/ Pro 300(3)+++/ pH 6/ SG 1.025/ Blo (+)/ Ket
5(0.5)/ Bil 1(17)+/ Glu (-)
Consultation results from immunology division on March 19th 2014:
Diagnosis : SLE
Advice : methylprednisolone injection 30 mg/kg/day given once a day, IVIG 4 g/kg/day,
hydroxychloroquin 6-7 mg/kg/day divided in 2 doses, & cyclophospamide 500-1000 mg/m2
Consultation results from nephrology division on March 20th, 22th, 25th & 26th 2014:
Diagnosis : lupus nephritis
Advice : CPA (20th), oral phenobarbital 60 mg 2 x 1 for 3 days & oral phenytoin 60 mg 2 x1 then
lowered the dose 20% (22th), CPA + mannitol 500-750 mg/m2 in 6 hours (25th),
metylprednisolone 4-4-4 (26th)
Laboratory findings on March 22th 2014:
Hemoglobin 9.7 gr/dL, Hematocrite 29.2%, Leucocytes 2.610/mm3, Platelet 147.000/mm3

Erythrocyte 3.41x106/mm3, Neutrophils 91.2%, Lymphocytes 6.1%, Albumin 2.3 g/dL

Ureum 63.9 mg/dL, Creatinine 0.66 mg/dL, Uric acid 11.1 mg/dL
Electrolyte Na 125 mEq/l, K 3.4 mEq/l, Ca 6.6 mg/dL

Discussion
A case control study in Sweden revealed that a history of hypertension, drug allergy, sun-reactive
skin type, smoking, transfusion, and a family history of SLE were all significantly associated
with an increasing risk of developing SLE.5 This patient did not admit having any of the risk
factors. Children most frequently present with fever, fatigue, hematologic abnormalities,
arthralgia or arthritis, rash, and renal disease. Symptoms may be intermittent or persistent.
People with SLE has cutaneous manifestations such as malar or butterfly rash which may be
photosensitive, discoid lesions, purpura, and many more. Arthralgia, arthritis, tendinitis, and
myositis are part of the musculoskeletal findings. Vasculitis, abdominal pain, diarrhea, infarction,
inflammatory bowel disease, hepatitis and hepatosplenomegaly are also commonly present.1
Serositis may mimic acute abdomen. The pathogenesis of serositis in SLE is considered
to be a vasculitis of the pleura or peritoneum caused by immune complex deposition and the
activation of complements.6 Cardiac involvement may affect all cardiac tissues with
manifestations that include valvular thickening, cardiomegaly, myocarditis, and even heart
failure. Pulmonary manifestations include pulmonary hemorrhage, infiltrates, and chronic
fibrosis. Early cardiopulmonary disease is often clinically silent. 1 Renal complications
(glomerulonephritis and microvascular thrombosis) and neuropsychiatric complications
(seizures, psychosis, neuropathies, stroke, and depression) are common as well. 2 MRI and CT
images may be normal even though abnormal neurologic manifestations are found.1
Diagnosis is based on classification criteria established by the American College of
Rheumatology (ACR). A minimum of 4 of the 11 ACR criteria should be met in order to qualify
as SLE.2 Elevated ANA titers are often present in children with active lupus. This is an excellent
screening tool, although ANA can be found without any disease or can be associated with
rheumatic, and other conditions, in either way, is far from specific. 1,7 The antinuclear antibodies
(ANA) test is highly sensitive with a positive result in >95% of SLE patients. 1 Significant titres
are accepted to be of 1:80 or greater.7

The anti-dsDNA antibody test is positive in 60-70% of SLE patients but in less than 0.5%
of healthy people or patients with other autoimmune diseases such as rheumatoid arthritis, thus it
is considered the best marker for serologic disease activity.1,2,8 Anti-dsDNA is more specific for
SLE, and its titres are also predictive of renal involvement. Moreover the titres of these
antibodies fluctuate with disease activity and therefore serial testing is a useful monitoring tool.
Typically, a disease flare is accompanied by a rising titre of dsDNA antibodies and erythrocyte
sedimentation rate (ESR), and falling complement and lymphocyte count.7 Both ANA and antidsDNA test were within the normal range on the first test done during her second day of
admission. This result put the SLE diagnosis in doubt at that moment. Then again, ANA
positivity is not mandatory for the diagnosis, and so the diagnosis of SLE can not be ruled out
yet.9 Surprisingly, the second test done on the 8th day of admission showed a seroconversion and
thus, this finding confirm the diagnosis of SLE on this patient.
In addition to blood work, renal biopsy is needed for those patients who develop renal
complications in order to classify severity.2 Oral ulcers,which is one of the eleven items on the
criteria suggested by ARC, is not found on the patient. Hematologic disorder on this patient is
manifested as a normochromic normocytic anemia, leucopenia, and a mild thrombocytopenia.
Neurological manifestation found in this patient is seizure preceeded by fever and organic
mental disorder during the first few days of admission. This patient does not have the
characteristic malar rash commonly found on people with SLE. Musculoskeletal finding was
arthralgia and also the main complain she had. Hepatomegaly, hepatitis, melena, abdominal pain
and diarrhea are often found. Renal involvement is proved by the abnormal value on the renal
function test and the gross hematuria found during the early course of the disease.
Patient was discharged on the March, 29 th and was suggested to get the disease controlled
routinely. Next visit was scheduled to be on April, 21st.
Conclusion
This paper reports a case of a 14 years old female diagnosed with Systemic Lupus
Erythematosus. A comprehensive work up had been done to confirm the diagnosis. The treatment
for this patient includes oral prednisolone for suppressing the autoimmunity, urdafalk
(ursodeoxycholic acid) for the hepatitis, zinc preparations, haloperidol, hydroxychloroquine, and

calnix (calcium carbonate). She was also given phenytoin & phenobarbital for suppressing her
seizure and after a while the drugs were tappered-off to oral dose for maintenance. While
furosemide and spironolactone given as advice from the cardiology consult are meant for
decreasing the preload as well as decreasing the work of the heart pumping blood with the defect
it has (mild MI).
References
1. Kliegman, R.M., Behrman, R.E., Jenson, H.B., and Stanton, B.F. Nelson Textbook of
Pediatrics, 19th ed. 2011. Saunders. Chapter 157.
2. Bailey, T., Rowley, K., and Bernknopf, A. A Review of Systemic Lupus Erythematosus and
Current Treatment Options. Formulary. 2011;46:178194.
3. Ringold, S., Lynm C., and Glass, R.M. Systemic Lupus Erythematosus. JAMA.
2005;293(24):3130.
4. Ginzler E, Tayar J. Systemic lupus erythematosus (lupus). Updated: January 2012. Available
at
http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/lupus.pdf#se
arch=sle. Accessed March 15, 2012.
5. Bengtsson, A.A., Rylander, L., Hagmar, L., Nived, O., and Sturfelt, G. Risk factors for
developing systemic lupus erythematosus: a casecontrol study in southern Sweden.
Rheumatology 2002;41:563-571.
6. Caltik, A., Demircin, G., Bulbul, M., Erdogan, O., Akyuz, S.G., and Arda N. An unusual
case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing
serositis and full-house nephropathy. Springer-Verlag. 2010.
7. Rahman, A. and Manson, J.J. Systemic Lupus Erythematosus. Orphanet Journal of Rare
Diseases. 2006, 1:6.
8. Isenberg DA, Shoenfeld Y, Walport M, et al. Detection of cross-reactive anti-DNA antibody
idiotypes in the serum of systemic lupus erythematosus patients and of their relatives.
Arthritis Rheum 1985;28:999-1007.
9. Berden JHM (1997) Lupus nephritis. Kidney Int 52:538558.