Systemic Lupus

Erythematosus
Supervisor: Dr. dr. Faridin HP, Sp.PD-KR
Radina Thahir
Nurul Arifah

Patient Identity
Name

: Ny. NK
Age
: 35 y.o
Date of admission : 22 December 2016
Address
: Sengkang
Job
: Teacher
Religion
: Islam
Race
: Bugis
Room
: Lontara 1 BD, 3/2/1
Medical record
: 782868

HISTORY TAKING
Chief complaint: weakness
History of present illness:

The patient was admitted to the hospital with

weakness that was felt since 2 weeks ago. The
pain is felt continuously and involves the whole
head. Patient has a history of intermittent fever
since 2 weeks ago, resolved in 2 days without any
additional medication. Patient has a history of
paling of fingers with numbness and occasional
pain which triggered by cold.
There is no history of seizure. There are no cough
and SOB. There are no nausea and vomiting. There
are no problems in defecation and urination.

Past medical history:

Patient had been diagnosed with Systemic
Lupus
Erythematosus
in
2013
by
a
rheumatologist, the symptoms were multiple
joint pain causing unability to walk; hair loss; red
rashes on face, arms, and legs when exposed to
sunlight; and frequent oral ulcer leading to
decrease of appetite. Red rashes are flat with no
scale, and persists on the cheeks. There was
also a frequent intermittent fever and poor
wound healing. Patient is on cell cept and
methylprednisolone regularly since then.
There is no family history with the same
complain.

Physical Examinations
general description :

Moderate illness/ deficient nutrition/ compos

mentis
Vital signs

BP : 110/70 mm/Hg
HR: 116 x/minute
RR : 22 x/minute
T : 36,6o C
Head :
Eye : pallor conjunctiva (+), Icteric scleric (-)
Face : Malar rash (+)
Mouth : oral ulcer (+)
Neck : DVS R+1 cmH2O

CHEST EXAMINATION
Inspection : symmetrical, no scar, ictus cordis
not visible
Palpation : no mass/tenderness, apex not
palpable
Percussion : normal heart size, liver border ICS 6
Auscultation :
Lung : vesicular breath sound
Rh -/- Wh -/Heart : S1 S2 regular, murmur (-)

ABDOMINAL EXAMINATION
Inspection

: Flat, follows breath movement

Auscultation : Peristaltic sound (+), normal
Palpation
: No mass/tenderness, liver and
spleen
are not palpable
Percussion
: Tympani (+), no ascites

RHEUMATOLOGY STATUS
Gait

: Normal
Arms :
Swelling (-), tenderness (-), erythem (-), warm (-),
crepitation (-)
Legs :
Genu dextra and sinistra :
Swelling (-), tenderness (-), erythem (-), warm (-),
crepitation(-)
Spine : Normal

LABORATORIUM
PARAMETER
RESULT
NORMAL

UNIT

VALUE
WBC

2.0

4.0 10.0

10^3 /uL

Lymph

0.29 (14.3%)

20.0 40.0

HGB

8.0

13.0 17.0

g/dL

RBC

2.76

4.0 6.0

10^6 /uL

RET

0.32

0.00 0.10

10^3 /uL

HCT

23

37.0 48.0

MCV

82

80-100

fl

MCH

29

27-32

pg

PLT

121

150 - 500

10^3 u/L

Ureum

31

10 - 50

mg/dL

Creatinine

1.25

<1,1

mg/dL

SGOT

38

<38

U/L

SGPT

<41

U/L

Glucose

72

<140

mg/dL

Protein

++

negative

mg/dL

CT SCAN
Consolidation in right
sphenoid sinus and
bilateral ethmoid sinuses;
impression: multisinusitis

PROBLEM LIST I
Systemic lupus erythematosus (SLE) based on :
Patient had been diagnosed with SLE in 2013
Malar rash
Hair loss
Hematologic disorder : leucopenia, lymphopenia.

NO.

DESCRIPTOR

SLEDAI
SCORE

Oral ulcer

Leucopenia

Total

3 (moderate)

therapy :
Glucocorticoid oral :
prednisone 5mg/24hours/oral
Mycophenolate mofetil :
500mg/12hours/oral
Topical sunscreen
SPF 15 at least; SPF 30 preferred

PROBLEM LIST II
Multi-sinusitis, based on:
Patient is suffering a headache since 2 weeks
ago.
CT Scan :
Consolidation in right sphenoid sinus and

bilateral ethmoid sinuses; impression:

multisinusitis
Plan :
Consult to Otolaryngologist

PROBLEM LIST III

Normocytic normochromic anemia, based on:
Conjunctiva pallor
Hb 8.0 mg/dL
MCV 82 fl
MCH 29 pg
Therapy:
Treat underlying cause
Plan:
Coombs test
Blood Smear Staining

PROBLEM LIST IV
Hypertension based on:
Blood pressure : 180/110 mmHg
Therapy :
Captopril 12,5mg/8hours/oral, decrease 20-25%

MAP
Plan :
consult to hypertension and nephrology division

Discussion

Definition
Systemic lupus erythematosus (SLE) is an

autoimmune disease in which organs and

cells undergo damage initially mediated by
tissue-binding autoantibodies and immune
complexes.

Epidemiology
Ninety percent of patients are women of child-

bearing years; people of all genders, ages,

and ethnic groups are susceptible.
Prevalence of SLE in the United States is 20 to
150 per 100,000 women depending on race
and gender.
Highest prevalence is in African-American and
Afro-Caribbean
women,
and
lowest
prevalence is in white men.

Etiology and Pathogenesis

Gene-environment

interactions result in
abnormal immune responses that generate
pathogenic
autoantibodies
andimmune
complexes that deposit in tissue, activate
complement, cause inflammation, and over
time lead to irreversible organ damage.

Etiology and Pathogenesis

Diagnosis and Clinical

Manifestations
SLICC Criteria for Classification of SLE

Diagnosis and Clinical

Manifestations
SLICC Criteria for Classification of SLE
Interpretation:Presence of
any 4 criteria (must have at
least 1 in each category)
qualifies patient to be
classified as having SLE with
93% specificity and 92%
sensitivity.
Note: Renal biopsy read as
systemic lupus qualifies for
classification as SLE even if
none of the other above
features are present.

Diagnosis Algorithm

Diagnosis
General
practitioners
(Primary Health
Center)

Suspect
SLE
Reconciliati
on

Continue &
Evaluate the
treatment
Continue &
Evaluate the
treatment

Reconciliati
on
Rheumatologist /
Internist
Mild SLE

Confirm diagnosis
Decide severity of
disease
Planning treatment

Moderate, Severe or Live threatening

SLE

Classification
Mild

Skin manifestation,
arthritis, organ system
function within normal
limits

Moderate

Found lupus nephritis mild to

moderate,
thrombocytopenia <50,000
and serositis major

Severe

Life-threathening,
thrombocytopenia <20,000,
severe nephritis, vasculitis
abdomen, severe anemia
hemolytic, massive pleural
and pericardial involvement,
significant renal damage,
CNS involvement

SLEDAI SCORE

MEX-SLEDAI SCORE

Management
There

is no cure for SLE, and complete

sustained remissions are rare, therefore the
physician should plan to induce remissions of
acute flares and then maintain improvements
with strategies that suppress symptoms to an
acceptable level and prevent organ damage.

Initial Treatment Algorithm

Treatments (Pharmacology)
Severity of SLE

Mild
Dermatology
manifestation
Arthiritis
Tx :
Hidroksiklorokuin /
Chloroquin or
Methotrexate

Cortocisteroid
NSAID

Moderate
Mild to moderate nefritis
Trombositopenia (trombosit
20-50x103/mm3)
Major serositis

Induction therapy
MP IV (0,5-1 gr / day for 3 `days
followed by :
AZA (2mg/kgBB/day) or MMF
(2-3 gr/day)
+
KS (0,5-0,6 mg/kg/day for 4-6
weeks and reduced gradualy

Maintenance therapy
AZA (1-2mg/kgBB/day) or MMF
(2-3 gr/day)
+
KS (KS reduced until 0,125
mg/kg/day interval

Severe
Severe nefritis with renal function test
Severe refracture trombositopenia
(trombosit < 20x103/mm3)
Severe refracture hemolitic anemia
Lung manifestation (hemorhagic)
NPSLE (cerebritis, mielitis)
Vaskulitis abdomen

Induction therapy
MP iv (0,5-1gr a day for 3 days)
+
CYC iv (0,5-0,75gr/m2/month x 7 doses)
Maintenance
therapy
CYC iv (0,5-0,75
gr/m2/3 months for a
year)

Enhanced Rituximab
Inhibitor Calcineurin
(Siklosporin)
IVIg (Imunoglobulin
intravena)

SLE Diagnosis
SJOGRENS
Differential
SYNDROME

RHEUMATOID
ARTHRITIS

DEFINITION

Chronic inflammatory
disease- autoantibody
response that involve
skin, joints, kidney,
blood cells and
nervous systems

Chronic inflammatory
disorder- lymphocytic
infiltrates in exocrine
organs- involve eye,
moth, parotid, glands
lungs, kidney, skin,
NS

Chronic systemic
autoimmune disease
that primarily involves
the joint.

ETIOLOGY

Autoimmune
Factor that induce Tcell intolerance
Sex hormone
Ultraviolet rays
Drugs
Infection

Autoimmune disease
Factor :
Virus

Autoimmune reaction
Genetic

EPIDEMIOLO
GY

Female > male

20-40 years old

Female > male

40-60 years old

35-50 years old

CLINICAL
MANIFESTATI
ON

Fever
Joint pain
rash

Dryness of eyes and

mouth
Xerostomia
Parotitis
Atralgia + arthritis
Vasculitis

Stiffness and
tenderness of joint
Vasculitis

LABORATORY

Specific antibody:
ANA
Anti ds-DNA
Anti-SM

Specific antibody :
Anti-Ro
Anti-La

Anti RF
Anti-CCP
Anti-MCV

Prognosis and Survival

Survival in patients with SLE in the United States, Canada,

Europe, and China is approximately 95% at 5 years, 90% at 10

years, and 78% at 20 years.
Poor prognosis (~50% mortality in 10 years) in most series is
associated with (at the time of diagnosis) high serum creatinine
levels (>1.4mg/dL), hypertension, nephrotic syndrome (24-h urine
protein excretion >2.6g), anemia (hemoglobin <12.4 g/dL),
hypoalbuminemia, hypocomplementemia, antiphospholipid
antibodies, male sex, ethnicity (African American, Hispanic with
mestizo heritage), and low socioeconomic status.
As many as 25% of patients may experience remissions,
sometimes for a few years, but these are rarely permanent.
The leading causes of death in the first decade of disease are
systemic disease activity, renal failure, and infections;
subsequently, thromboembolic events become increasingly
frequent causes of mortality.

THANK YOU