SYSTEMIC LUPUS ERYTHEMATOSUS

Presented by : Jackvinderdeep Singh A/L Taram Singh

Evelin Simarmata
Hari/tanggal :
Surpervisor : dr . Rita Evalina Rusli, M.Ked(Ped), Sp. A(K)

Introduction
Systemic Lupus Erythematosus ( SLE) is a chronic (long term) autoimmune diseases
with a heterogeneous presentation. Systemic Lupus Erythematosus ( SLE) is a
chronic (long term) autoimmune diseases with a heterogeneous presentation. The
word systemic is used because it can affect so many organs and tissues in the body
meanwhile lupus refers to the rash that extends across the bridge of the nose and
upper checkbones and was thought to resemble a wolf bite. The word erythematosus
refers to the colour of the rash which is in red in colour1.
Systemic lupus erythematosus is a multisystem disease which is characterized
by an autoantibody response to nuclear and cytoplasmic antigens. Lupus can be mild
to severe and affects mostly women. Lupus may affect various parts of the body but it
mostly manifests in the skin , joints, and kidneys. It was first described in 1828
whereby over 90% of patients with SLE have positive anti nuclear antibodies (ANA).
This disease is a relapsing and a remitting one.3
Lupus has no single diagnostic marker but instead is identified through a
combination of clinical and laboratory criteria. Accurate diagnosis of SLE is
important because treatment can reduce mortality and morbidity.The diagnosis of
SLE is basicly made upon clinical findings that comprises of the presence of 4 out
of 11 criteria such as malar rash, discoid rash, photosensitivity, oral ulcers, arthritis,
serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic

disorder, and antinuclear antibody. Lupus has many different symptoms but the
common ones include fatigue, joint pain and skin rashes4.
There is no cure for lupus. Treatment depends on the type of symptoms and
how serious the disease is. The treatment usually uses medications like NSAID,
antimalarial drugs, corticosteroids, and immune suppressants.5
The aim of this paper is to report a case of Systemic Lupus Erythematosus in a
9year and 7 months old Indonesian girl.
Case
JFS, 9 years and 7 months old Indonesian girl was admitted on the February , 28 th
2015 with a chief complaint of fever. The patient had fever for almost 10 days before
the day of admission. A high grade fever, mostly at evening and night and shivering
was notified (+) together with no convulsion (-). The fever was believed to have
subside upon the use fever relieving medicine. The patient also had cough (+) with
phlegm(+) for a duration of 7 days with no record of vomiting(-) and diarrhea(-) or
neither stomachache( -).Patient had a normal urination(+) and defecation(+) with n
abnormalities. History of past illness of patient was tat she was referred by a child
specialist from Mitra Sejati Hospital with a diagnoses of thypoid fever and
bronchopneumonia. Among the medicines that the patient has consumed were
Paracetamol, Ambroxol, Mullavit, Cefixime, Cetirizine, Hidrocortison Cream.
Physical Examination
General status
Body weight: 24 kg, Body length: 120 cm,
Body weight in 50th percentile according to age: 31 kg
Body length in 50th percentile according to age: 137 cm
Body weight in 50th percentile according to body length: 22 kg
BW/BL: 30,5/38 x 100% = 109,09%
BW/age: 24/31 x 100% = 77,41%

BL/age : 120/137 x 100% = 87,59%

Presens status
Consciousness: GCS 15 (E4 V5 M6)
Blood Pressure: Heart Rate: 82 x/i
Respiratory Rate: 20 x/i
Body Temperature: 38,3 oC.
Anemic (+). Icteric (-). Cyanosis (-). Edema (-). Dyspnea (-).
Local status
Head :
Eyes

: Isochoric pupil 3 mm. Inferior palpebra conjunctiva pale (-/-).

Icteric sclera (-/-). Light reflex (+/+). Face edema (-).Inferior and superior palpebra
edema (-/-).
Ears and nose : Within normal limit
Mouth

: Pale mucosa (-), thypoid tongue (+), oral ulcer (+)

Neck :
Lymph node enlargement (+), right = 4 nodes, ( 1 x 1 cm), left = 3 nodes ( 1 x 1
cm), immobile
Thorax:
Symmetric fusiformis. Chest retraction (-). HR: 82 x/i, regular, murmur (-). RR: 24
x/i, reguler, ronchi (-). Breath sound: vesicular. Additional sound (-).
Abdomen:
Soepel.Liver and spleen unpalpable, Skin pinch returns quickly, Shifting dullness (-),
Double sound (-), Ascites (-)
Extremities:

Pulse 82 x/i, regular, adequate pressure and volume, warm, CRT < 3. Pitting edema
(-), Lupus Discoid (+)
Skin:
Macula erythematosus, hyperpigmentation on face, chest, back, and on upper arm of
both hands. Arcus silialis silialis (+), skuama (+), erythema (+) on oral mucosa and
lips, white patches on buccal (+) and malar rash (+)
Differential Diagnosis:
-

Thypoid fever

Urinary track infection

+ drug eruption

Working Diagnosis:
-

Thypoid fever

Management:
-

IVFD D5% NaCl 0,45% 50 gtt/i micro

Inj. Ceftriaxone 1 gr/12 hrs/IV
PCT 3 x 300 mg
CTM 3 x tab
Diet with heavy food 2400 Kkal + 48 gr protein

Diagnostic Planning:
-

Complete urinalysis
Blood clinical chemistry
Consult to allergic and immunologic specialist

Laboratory finding :
February, 28th 2015
(Urinalysis)
-All components like colour, glucose levels, bilirubin levels, keton and others were in
normal values expect for there was blood (+) and protein (++) in the urin.
FOLLOW UP

March, 2nd 2015

S Fever (+)
O Cons: alert, Temp: 38,2 oC
Body weight: 24 kg, Body length: 120 cm.
Head

Isochoric pupil 3 mm. Inferior palpebra conjunctiva pale (-/-). Icteric

sclera (-/-). Light reflex (+/+). Face edema (-).Inferior and superior
palpebra edema (-/-).
Ears and nose : Within normal limit

Neck

Mouth
: Pale mucosa (-), oral ulcer(+)
Lymph node enlargement (+), right = 4 nodes, ( 1 x 1 cm), left = 3

Thorax

nodes ( 1 x 1 cm), immobile

Symmetric fusiformis. Chest retraction (-). HR: 88 x/i, regular, murmur
(-). RR: 22 x/i, reguler, ronchi (-). Breath sound: vesicular. Additional

Abdomen

sound (-)
Soepel.Liver and spleen unpalpable, Skin pinch returns quickly, Shifting

dullness (-), Double sound (-), Ascites (-)

Extremities Pulse 82 x/i, regular, adequate pressure and volume, warm, CRT < 3.
Skin

Pitting edema (-)

Macula erythematosus, hyperpigmentation on face, chest, back, and on
upper arm of both hands. Arcus silialis (+), skuama (+), erythema (+) on
oral mucosa and lips, white patches on buccal (+), Malar rash (+), discoid

A
P

rash (+).
Thypoid fever + drug eruption + DD/ - LupusManagement:
-

IVFD D5% NaCl 0,45% 50 gtt/i micro

Azithromycin 1x 2 cth
PCT 3 x 300 mg
CTM 3 x tab
Diet with heavy food 2400 Kkal + 48 gr protein

Diagnostic Planning:
-

Full blood count

Blood gas analysis
Tubex
Widal test

ANA test
Anti ds DNA test
March, 3rd 2015

S Fever (+)
O Cons: alert, Temp: 38,2 oC
Body weight: 24 kg, Body length: 120 cm.
Head

Isochoric pupil 3 mm. Inferior palpebra conjunctiva pale (-/-). Icteric

sclera (-/-). Light reflex (+/+). Face edema (-).Inferior and superior
palpebra edema (-/-).
Ears and nose : Within normal limit

Neck

Mouth
: Pale mucosa (-), oral ulcer (+)
Lymph node enlargement (+), right = 4 nodes, ( 1 x 1 cm), left = 3

Thorax

nodes ( 1 x 1 cm), immobile

Symmetric fusiformis. Chest retraction (-). HR: 88 x/i, regular, murmur
(-). RR: 22 x/i, reguler, ronchi (-). Breath sound: vesicular. Additional

Abdomen

sound (-)
Soepel.Liver and spleen unpalpable, Skin pinch returns quickly, Shifting

dullness (-), Double sound (-), Ascites (-)

Extremities Pulse 82 x/i, regular, adequate pressure and volume, warm, CRT < 3.
Skin

Pitting edema (-)

Macula erythematosus, hyperpigmentation on face, chest, back, and on
upper arm of both hands. Arcus silialis (+), skuama (+), erythema (+) on
oral mucosa and lips, white patches on buccal (+), malar rash (+), discoid

A
P

rash (+).
- Lupus Management:
-

IVFD D5% NaCl 0,45% 50 gtt/i micro

Azithromycin 1x 2 cth
PCT 3 x 300 mg
Diet with heavy food 2400 Kkal + 48 gr protein

Diagnostic Planning:
-

Complete Blood Count

Liver Function Test
Serum Electrolytes

Laboratory Findings: March, 2nd 2015

-Blood gas analysis were all normal expect for PCO2 which was low ( 20mmHg),
bicarbonat (HCO3) (14.2 mmol/L) and the base escess ( -8.3 mmol/L) that were low.
-Electrolytes were all in normal ranges expect for calcium (Ca) which was low
(7.1mg/dl) and natrium (Na) which was also below the normal limit (117mEq/L).
-Widal test recorded all normal findings.
-Tubex test showed normal findings.
-Complete Blood Count showed all was within the normal values expect for the
hemoglobin

(10.1

gr%),

hematocrite

(29.1%),

leukocyte

and

thrombosit

(114.000/mm3) that were low.

Peripheral blood smear: Normochromic normocytic anemia + Leukopenia +
thrombocytopenia
March, 4 - 7th 2015
S Fever (-)
O Cons: alert, Temp: 37 oC
Body weight: 24 kg, Body length: 120 cm.
Head

Isochoric pupil 3 mm. Inferior palpebra conjunctiva pale (-/-). Icteric

sclera (-/-). Light reflex (+/+). Face edema (-).Inferior and superior
palpebra edema (-/-), butterfly rash (+)
Ears and nose : Within normal limit
Mouth

: Pale mucosa (-), ulcer (+), white patches (+) on buccal,

Neck

erythema on lips (+)

Lymph node enlargement (+), right = 4 nodes, ( 1 x 1 cm), left = 3

Thorax

nodes ( 1 x 1 cm), immobile

Symmetric fusiformis. Chest retraction (-). HR: 90 x/i, regular, murmur
(-). RR: 20 x/i, reguler, ronchi (-). Breath sound: vesicular. Additional

Abdomen

sound (-)
Soepel.Liver and spleen unpalpable, Skin pinch returns quickly, Shifting

dullness (-), Double sound (-), Ascites (-)

Extremities Pulse 90 x/i, regular, adequate pressure and volume, warm, CRT < 3.
Skin
A
P

Pitting edema (-), fenomena Reynoud (-)

Macula erythematosus, hyperpigmentation on face, chest, back, and on

upper arm of both hands. Arcus silialis (+), skuama, discoid rash (+).
- Systemic Lupus Erythematosus
Management:
-

IVFD D5% NaCl 0,45% 50 gtt/i micro

Inj. Methylprednisolon (30 mg/KgBB/day) = 750 mg/IV/day, in 100 cc NaCl 0,9 % -

finished in 1 hour, for three days

Inj. Ranitidin 25 mg/ 8 hr/IV
PCT 3 x 300 mg
CTM 3 x tab
Diet with heavy food 1600 kalori
Methylprednisone oral 1-1-1 (0.5mg/kg/BB/hr)
Parasol cream s.u.e

Diagnostic Planning:
- Full Blood Count
- Blood Electrolytes
- Echocardiography,
Echocardiography
Result = Normal
March, 8 - 9th 2015
S Small rash on face (+), athralgia (+)
O Cons: alert, Temp: 36,9 oC
Body weight: 24 kg, Body length: 120 cm.
Head

Isochoric pupil 3 mm. Inferior palpebra conjunctiva pale (-/-). Icteric

sclera (-/-). Light reflex (+/+). Face edema (-).Inferior and superior
palpebra edema (-/-).
Ears and nose : Within normal limit

Neck

Mouth
: Pale mucosa (-), oral ulcer (+)
Lymph node enlargement (+), right = 4 nodes, ( 1 x 1 cm), left = 3

Thorax

nodes ( 1 x 1 cm), immobile

Symmetric fusiformis. Chest retraction (-). HR: 82 x/i, regular, murmur
(-). RR: 22 x/i, reguler, ronchi (-). Breath sound: vesicular. Additional

Abdomen

sound (-)
Soepel.Liver and spleen unpalpable, Skin pinch returns quickly, Shifting

dullness (-), Double sound (-), Ascites (-)

Extremities Pulse 82 x/i, regular, adequate pressure and volume, warm, CRT < 3.
Skin

Pitting edema (-)

Macula erythematosus, hyperpigmentation on face, chest, back, and on
upper arm of both hands. Arcus silialis (+), skuama (+), erythema (+) on

A
P

oral mucosa and lips, white patches on buccal (+)

- Systemic Lupus Erythemathosus
Management:
-

IVFD D5% NaCl 0,45% 50 gtt/i micro

CPA (500 1000 mg/m2) = 450 mg, in 250 cc NaCl 0,9 % given along with Mesna 90

mg, in 100 cc NaCl 0,9 %, every 8 hours finished in 1 hour, for 1 day
Inj. Ranitidin 25 mg/ 8 hr/IV
Methylprednisolon oral 1-1-1 (0.5mg/kg/bb/hr)

Diagnostic Planning:
-

Antasida syr 3 x cth I

Parasol cream s.u.e.
Kenalog for mouth
Diet 1580 kkal + 48 gr protein

Laboratory Findings: March, 7th 2015

LIVER FUNCTION
-Normal
ELECTROLYTES
-All were in normal values
Laboratory Findings: March, 9th 2015
-All are in normal values expect for hemoglobin (10.7 gr%) and hematocrite (32.1
%) that were low.
Peripheral blood smear: Normochromic normocytic anemia

Discussion
Lupus is a chronic inflammatory disease that occurs when your own body immune
system attacks your own tissues and organs. Inflammation caused by lupus can affect
many different body systems including your joints, skin, kidneys, brain and heart. It
can be mild to severe.There are many possible causes of SLE and current studies have
found that immune system dysfunction factors, genetic factors, environmental factors,
and hormonal factors do play an imporrtant role in provoking SLE.1
The pathogenesis of this disease starts when there is an immune responses
against endogenous nuclear antigens. Autoantigens released by apoptotic cells are
presented by dendritic cells to T cells leading to their activation. Activated T cells in
turn help B cells to produce antibodies to these self-constituents by secreting
cytokines such as interleukin 10 (IL10) and IL23 and by cell surface molecules such
as CD40L and CTLA-4. In addition to this antigen-driven T cell-dependent
production of autoantibodies, recent data support T cell-independent mechanisms of
B cell stimulation via combined B cell antigen receptor (BCR) and TLR signalling.
The pathogenesis of SLE involves a multitude of cells and molecules that participate
in apoptosis, innate and adaptive immune responses (table 1).Increased amounts of
apoptosis-related endogenous nucleic acids stimulate the production of IFN and
promote autoimmunity by breaking self-tolerance through activation of antigenpresenting cells. Once initiated, immune reactants such as immune complexes
amplify and sustain the inflammatory response.6
The patient was first diagnosed with thypoid fever which wasnt confirmed
and then with lupus due to the presence of 4 out of 11 criteria such as malar rash,
discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder,
neurologic disorder, hematologic disorder, immunologic disorder, and antinuclear
antibody. Serological biomarkers hold a significant potential in diagnosis and
monitoring of SLE given that the pathogenesis is most likely the result of immune
dysregulation. Anti-double -stranded DNA (anti-dsDNA) is highly specific for lupus,
with 70% of SLE patients being positive in comparison with only 0.5% of the healthy
population or those with other autoimmune diseases. In contrast, antinuclear antibody

is highly sensitive, being positive in 99% of SLE patients at some point in their
illness, but is also found in 32% of the general population at a 1:40 dilution and in 5%
at a 1:160 dilution.The search for biomarkers with higher sensitivity and specificity
continues with flow cytometric analysis of erythrocyte-bound complement activation
product C4d and complement receptor giving promising results. Despite advances it
must be recognized that there is no definitive laboratory test for the diagnosis or
monitoring of SLE and that all results must be viewed in the context of each
individual patients clinical course.4
Differential diagnosis from other polyarticular diseases affecting young
women, such as rheumatoid arthritis or Stills disease, may not be easy at the initial
stages. Other diseases to be considered include undifferentiated connective tissue
disease,

primary

Sjogrens syndrome,

primary

antiphospholipid

syndrome,

fibromyalgia with positive ANA, idiopathic thrombocytopenic purpura, drug induced

lupus, and autoimmune thyroid disease. Patients presenting with fever or
splenomegaly and lymphadenopathy must be differentiated from infectious diseases
or lymphoma. In febrile patients with known SLE, leucocytosis, neutrophilia, shaking
chills, and normal levels of anti-DNA antibodies favour infection. Lupus may present
with localised or generalised lymphadenopathy or splenomegaly, but the size of
lymph nodes is rarely >2 cm while splenomegaly is mild-to-moderate. Patients with
known or suspected SLE with prominent lymphadenopathy, massive splenomegaly or
expansion of a monoclonal CD19+/ CD22+ B cell population should raise the
suspicion of non-Hodgkin lymphoma.6
In

patients

presenting

with

neurological

symptoms,

infections,

cerebrovascular accidents or immune mediated neurologic diseases such as multiple

sclerosis or Guillain-Barre disease must be considered. Finally, in patients presenting
with pulmonaryrenal syndrome, the disease must be differentiated from
Goodpastures syndrome, or antineutrophil cytoplasmic antibody (ANCA) associated
vasculitis.6

There is no cure for SLE. Treatment depends on the type of symptoms and how
serious the disease is. The treatment usually uses medications like NSAID,
antimalarial drugs, corticosteroids, and immune suppressants.2 Current treatment of
SLE has improved survival rate dramatically, prolonged and complete remission
defined as 5 years without clinical and laboratory evidence of active disease.6
Conclusions
It has been reported, a young girl, 9 years 7 months old with the main complaint fever
and was diagnosed with SLE. The diagnose was established based on history taking,
clinical manifestations, and laboratory finding. The patient got Methylprenidsolon
injection for three days and ranitidin injection, followed by cyclophospamide
chemotherapy for 1 day. Then the patient got methylprenidsolon oral 1-1-1 along with
antasida, parasol cream, and kenalog for mouth and still need to be followed up.
References

1. Anon. The American College of Rheumatology nomenclature and case

definitions for neuropsychiatric lupus syndromes. Arthritis Rheum
1999;42:599608.
2. Bertsias G, Ioannidis JP, Boletis J, et al. EULAR recommendations for the
management of systemic lupus erythematosus. Report of a Task Force of
the EULAR Standing Committee for International Clinical Studies
Including Th erapeutics. Ann Rheum Dis 2008;67:195205.
3. Cervera R, Khamashta MA, Font J, et al. Systemic lupus erythematosus:
clinical and immunologic patterns of disease. The European Working
Party

on

Systemic

Lupus

Erythematosus.

Medicine

(Baltimore)

1993;72:11324.
4. Edworthy SM. Clinical manifestations of systemic lupus erythematosus.
In: Ruddy S, Harris ED, Sledge CB, Kelley WN, eds. Kelleys Textbook
of rheumatology. 6th ed. Philadelphia: Saunders, 2001:1105-19.

5. Guidelines for referral and management of systemic lupus erythematosus

in adults. American College of Rheumatology Ad Hoc Committee on
Systemic

Lupus

Erythematosus

Guidelines.

Arthritis

Rheum

1999;42:1785-96.
6. Robert M. Kliegman, MD, Bonita M.D. Stanton, MD, Joseph St Geme,
Nina Schor, MD, Phd. Nelson Textbook Of Pediatrics Diseases 19th
Edition.