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.
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