LUPUS ERITEMATOSUS

SISTEMIK

Raveinal
Subbagian Alergi Imunologi Klinik
Bagian Ilmu Penyakit Dalam FK UNAND / RS M Jamil
Padang

Lupus eritematosus sistemik (LES)

Penyakit autoimun yang melibatkan berbagai organ

dengan manifestasi klinis yang bervariasi dari yang

ringan sampai berat .
Pada keadaan awal, sering sekali sukar dikenal sebagai
LES, karena manifestasinya sering tidak terjadi
bersamaan.
Penyebab pasti belum diketahui, ada dugaan faktor
genetik, infeksi dan lingkungan ikut berperan pada
patofisiologi LES

Rasio : (6 7)
Wanita

1
laki-laki

Steps in Pathogenesis of SLE

1.
2.
3.
4.
5.
6.
7.

Genetic factors/immune dysfunction

Environmental/endogenous trigger
Inflammation
Development of Autoimmune
Accelerated of Antigen
Tissue Damage
Clinical Disease

Autoimmune pathogenesis
AUTOIMMUNE
*DIATHESIS*
Genetic Risk Factors :
- MHC
- Other
Trigger
Factor
(Infective
Ischaemic
latrogenic)

AUTOIMMUNITY
WITHOUT DISEASE
Autoreactive
Lymphocytes

EVOLVING
SPECTRUM OF
AUTOIMMUNE
DISEASE

Tolerance Breakdown
Autoimmune Diseas
- APC stimulation
Susceptible End - Lupus
- Molecular mimicry
Organs
- Sjrgrens
- Cryptic antigen recognition (Skin, joint,
- Scleroderma
- Immune / apoptotic
others)
- Rheumatoid
- dysregulation
- Polymyositis

Autoreactive
Lymphocytes

Autoreactive
Lymphocytes

ulating and Perpetuating Factors acting at multiple levels: e.g. UV radiation, hormones, dru
Reeves G.E.M. Update on the immunology, diagnosis and management of systemic lupus erythematosus. Internal Medicine Journal 2004;34:338-347

Autoantibodies
Autoantibodies noted
noted in
in SLE
SLE and
and their
their clinical
clinical relevance
relevance
Side
Side

Antigen
Antigen

Clinical
Clinical features
features

Nucleus
Nucleus

ds-DNA
ds-DNA
ss-DNA
ss-DNA
U1-RNP
U1-RNP

Characteristic
Characteristic of
of SLE/nephritis
SLE/nephritis
Non-specific
Non-specific
Present
Present in
in aa variety
variety of
of connective
connective tissue
tissue
disease
disease including
including SLE,
SLE, mixed
mixed connective
connective
tissue
tissue disease
disease and
and overlap
overlap syndromes
syndromes
(myositis,
(myositis, sclerodactyly,
sclerodactyly, Raynaud's)
Raynaud's)
Characteristic
Characteristic of
of SLE
SLE
Present
Present in
in drug-related
drug-related lupus
lupus
Presence
Presence associated
associated with
with decreased
decreased
nephritis
nephritis
Present
Present in
in aa number
number disorders,
disorders, including
including
SLE,
SLE, Sjogrens
Sjogrens syndrome,
syndrome, congenital
congenital heart
heart
block,
block, neonatal
neonatal lupus
lupus
Presence
Presence characteristic
characteristic of
of SLE
SLE

Sm
Sm
Histones
Histones
SSB/La
SSB/La
SSA/Ro
SSA/Ro
PCNA
PCNA
Ribosomal P-protein
P-protein
Cytoplasm
Cytoplasm Ribosomal
Red
Red cells
cells
Cell
Cell
White celts
celts
membranes
membranes White
Platelets
Platelets
Phospholipid
Phospholipid

Psychosis,
Psychosis, depression
depression
Coomb's
Coomb's positive
positive haemolytic
haemolytic anemia
anemia
Lymphopenia
Lymphopenia
ThrombocytopeniaThrombocytopeniaThrombosis,
Thrombosis, recurrent
recurrent abortions
abortions

Mekanisme pengendapan imun kompleks

Vasculitis

Gambaran klinis LES

Limphadenopati
Limphadenopati
12-50%
12-50%
SSP
SSP
20%
20%
Hepotomepali/
Hepotomepali/
Splenomegali
Splenomegali
20%
20%
Sal cerna
18%
18%

Kelelahan
Kelelahan
90%
90%
Panas
Panas lama
lama
80-82%
80-82%
BB
BB turun
60%
60%
Artritis/Artralgia
Artritis/Artralgia
90%
90%
Kulit
Kulit
50-58%
50-58%

LES

Paru
Paru
38%
38%
Hematologi
Hematologi
50%
50%

Jantung
Jantung
48%

Vaskulitis
Vaskulitis

Ginjal
Ginjal
50%

Faktor pencetus/eksaserbasi
Obat
Obat ::

Procainamid
Procainamid
Hidralazin
Hidralazin
Metildopa
Metildopa
CPZ
CPZ

Keguguran
Keguguran

Kehamilan
Kehamilan

Sinar
Sinar UV
UV
(320-400
(320-400 nm)
nm)

LES

Infeksi
Infeksi

Tindakan
Tindakan
pembedahan
pembedahan

Kriteria ARA
1.
2.
3.
4.
5.
6.

Ruam Malar
Ruam Diskoid
Foto sensitif
Ulkus di mulut
Artritis/artralgia
Serositis
a. Efusi perikardial
b. Efusi pleura
7. Kelainan ginjal
a. Proteinuria (>0.5 gr//24 jam)
b. Cellular cast

Kriteria ARA
8. Kelainan neurologis
9. Kelainan darah
a. Anemia hemolitik
b. Leukopenia (< 4000)
c. Limfopenia (<1500)
d. Trombositopenia (<100.000)
10. Sero-imunologi
a. Anti ds DNA
b. Anti Sm
11. ANA

Small Vessel
Vasculitis

Discoid Lupus

Oral Ulcers

Photosensitivity

Discoid Lupus

Erythematous Rash

P EN ATA LA K S A N A A N S LE

Kelelahan

Sinar matahari

Diet
P
enatalaksanaan
Penatal
U m um

Cuaca

Merokok
Kontrasepsi oral

Stres dan
trauma fisik

Pengobatan LES
Kortikosteroid

- Prednison
- Metilprednisolon

Imunosupresif
- Azathioprin
- Metotreksat
- Siklofospamid
Anti malaria
- Hidroksikloroquin
- Kloroquin
Plasmaferesis
Imunoterapi
- IVIG
- Imunoabsorpsi ds DNA
- Monoklonal antibodi

Pengobatan m uskuloskeletalpada LES

Artritis Terdinitis
Myositis Artralga/
myalgia
NSAID
+
(+)
Anti malaria
+
Steroid
(+)
Imunosupresan -

(+)
-

+
(+)

44 // 11
11

ARA

33 // 11
11

DOSIS STEROID

LES
Kerusakan
Kerusakan
Organ
Organ
Vaskulitis
Vaskulitis

60-100
60-100 mg/hari
(Hari-minggu)
(Hari-minggu)

Ginjal
Ginjal

Hematologi
Hematologi

Jantung
Jantung

Paru
Paru

Anemia
Efusi
Anemia hemolitik
hemolitik
Efusi
1-1,5
15-40
1-1,5 mg/kgbb/hari
15-40 mg/hari
mg/hari
60-80mg
60-80mg
100-120 mg ++ drainage
drainage
(4-6
(4-6 minggu
minggu // 8-12
8-12 minggu)
minggu) L.pneumonitis
L.pneumonitis
1-1,5
1-1,5 mg/kg/hari
mg/kg/hari
Trombositopeni
Trombositopeni
(4-6
(4-6 minggu)
minggu)
1-2
1-2 mg/kgbb/hari
mg/kgbb/hari
60-80
60-80 mg/hari
(4
(4 minggu)

SSP
SSP

Metilpred
Metilpred
22 mg/kg/hari
mg/kg/hari
3-5
3-5 hari
hari

oral
oral pred
pred
48-80
48-80 mg/hari
mg/hari
5-7
5-7 hari
hari

LES

Inaktif
Inaktif

NL
50-60%

APS

Nepritis
Nepritis lupus(NL)
lupus(NL)
Hipertensi
Hipertensi

KEHAMILAN

Preleklamsi
Preleklamsi

Sc
Sc (+)
(+)

Abortus
Abortus

Sc
Sc (-)
(-)

DPL
DPL
Urinalisis
Urinalisis
Ureum/Cr/GD
Ureum/Cr/GD
ACL/LA
ACL/LA
SSA/SSB
SSA/SSB
C3/C4
C3/C4
Antids
Antids DNA
DNA
24
24 jam
jam Urin
Urin
protein
protein

PARTUS
Normal
Normal

Marformasi
Marformasi
kongenital
kongenital
Pred
Pred (-)
(-)
Sitotoksik
Sitotoksik (+)
(+)

C.Heart
C.Heart Block
Block
8,8%
8,8% SSA,SSB
SSA,SSB (+)
(+)
1%
1% SSA,SSB
SSA,SSB (-)
(-)

Prematur
Prematur
60%
60%

IUGR
IUGR
30%
30%

PULSE DOSE METHIL PREDNISOLON

1 gr/IV 3 hari berturut-turut

Oliguria akut (RF)

Lupus serebral dengan koma
Krisis lupus (acute serious SLE)

TERIMA KASIH