SYSTEMIC LUPUS

ERITHEMATOSUS
Disusun Oleh : Asy Syifa Dhiya Ulhaqq Anwar (629222012)

Dokter dr. Nurhidayah, Sp. A

Pembimbing :
 Definisi

Lupus Eritematosus Sistemik (LES) atau Lupus merupakan penyakit autoimun kompleks
yang menyerang berbagai sistem dalam tubuh, mengenai satu atau beberapa organ tubuh,
seperti ginjal, kulit, sel darah dan sistem saraf yang ditandai dengan adanya inflamasi luas
pada pembuluh darah dan jaringan ikat yang bersifat episodik diselingi oleh periode remisi.
 Epidemiologi
• Prevalensi lupus bervariasi pada tiap negara dengan angka kejadian lupus empat kali lebih
banyak terjadi pada afrikan-amerikan dibandingkan kaukasian.

• Angka insidensi lupus di Asia Pasifik sebesar 0,9-3,1/100.000 penduduk setiap tahunnya.

• Di Indonesia, insidensi lupus pada anak secara umum mengalami peningkatan, yaitu sekitar
15-17%.

• Lupus jarang terjadi pada anak dengan usia di bawah 5 tahun.

• Penyakit ini paling sering mengenai anak perempuan dengan usia 9-15 tahun dengan ratio
perempuan dan laki-laki adalah 2:1 sebelum pubertas dan setelah pubertas menjadi 9:1.
 Etiologi

Faktor
01 Faktor Genetik Lingkungan 02

03 Faktor Hormonal Faktor Imunologi 04

Patofisiologi

5
Etiologi
 Manifestasi Klinis

Tahap Awal (Konstitusional)

• Kelemahan pada tubuh

• Penurunan nafsu makan

• Berat badan menurun

• Demam tanpa fokus infeksi

• Limfadenopati (generalisata maupun lokal)

 Manifestasi Klinis

Tahap Lanjutan (Sistemik) • Jantung

• Muskuloskeletal • Ginjal

• Kulit dan mukosa • Gastrointestinal

• Hematologi • Mata

• Neuropsikiatri

• Paru
 Pemeriksaan Fisik
• Tanda-tanda rambut mudah dicabut atau terdapat area kebotakan

• Konjungtiva anemis

• Bercak-bercak kemerahan di wajah (butterfly rash)

• Discoid rash pada alis

• Pemeriksaan thorax  Nyeri dada tanpa kelainan radiologi yang nyata, sesak napas, hemoptisis, dan demam. Pada
keadaan berat, dapat ditemukan efusi pleura

• Pemeriksaan abdomen  Nyeri tekan pada abdomen yang menandakan adanya vaskulitis dari pembuluh darah
usus

• Pada pemeriksaan ekstremitas  Pemeriksaan nyeri tekan pada persendian.

• Pada pasien anak perlu dilakukan perhitungan status gizi berdasarkan tabel pengukuran CDC 2000 BB/U, TB/U, dan
BB/TB.
 Pemeriksaan Penunjang

Darah Lengkap Imunologi

Anemia, leukopenia, limfopenia Antibodi Antinuklear (ANA), Antibodi
trombositopenia, dan kadar LED yang anti-double stranded DNA (anti-dsDNA),
meningkat dan Antibodi anti-Smith (anti-Sm)

Urine Lengkap Kadar Komplemen

Proteinuria, hematuria, piuria, dan Penurunan C3 dan C4
kelainan sedimen (cast urin, dan cast
eritrosit)
 Pemeriksaan Penunjang
Pemeriksaan Imunologi stranded DNA (anti-dsDNA)
1. Antibodi Antinuklear (ANA) • Bagian dari ANA, dapat dilakukan dengan beberapa
• Pemeriksaan pertama yang diperlukan bagi setiap metode yaitu ELISA, farr assay, dan Crithidia luciliae
pasien dengan kecurigaan lupus immunofluorescence test (CLIFT)
• Dapat dilakukan dengan indirect • Sangat spesifik untuk lupus, oleh karena itu anti-
immunofluorescence (IIF) dan enzyme-linked dsDNA sangat membantu dalam menegakkan
immunosorbent assay (ELISA) diagnosis yang pasti
2. Antibodi anti-double stranded DNA (anti-dsDNA) 3. Antibodi anti-Smith (anti-Sm)
• Hasil titer ANA ≥1:40, maka dapat dipertimbangkan • Memiliki spesifisitas yang tinggi untuk lupus
tes yang lebih spesifik seperti Antibodi anti-double
12
Diagnosis
American Criteria Rheumatology/European League Against
Rheumatism 2019 (ACR/EULAR)

Keterangan penggunaan kriteria ACR 2019, yaitu :

• Riwayat ANA-IF positif ≥ 1:80 (diagnosis ditegakkan dengan
melihat kriteria tambahan yang mungkin ditemukan)
• Skor pada kriteria tidak dihitung bila ada sebab selain lupus
• Kemunculan kriteria cukup satu kali
• Hanya mengambil skor tertinggi tiap domain untuk skor total
• Klasifikasi lupus pada skor total ≥ 10, minimal satu kriteria klinis

13
 Penatalaksanaan

Anti Inflamasi Non Steroid (AINS) Anti Malaria

• Naproksen : Biasa digunakan pada anak dengan• Hidroksiklorokuin : Dosis inisial 6-7 mg/kg
dosis 10-20 mg/kgBB/hari po, dibagi 2-3 dosis BB/hari dibagi 1-2 dosis selama 2 bulan,
• Tolmetin Sodium (Tolektin) : Biasa digunakan dilanjutkan 5 mg/kgBB/hari p.o. (maks.300
pada anak dengan dosis 20-30 mg/kgBB/hari, mg/hari)
po, dibagi 3-4 dosis • Kuinakrin : Dosis dewasa: 100 mg/hari
• Salisilat : Anak <20 kg: dosis 80–90• Klorokuin HCl: Dosis dewasa: 250 mg/hari
mg/kgBB/hari po dibagi 3-4 dosis, anak >20 kg:
dosis 60–80 mg/kgBB/hari po dibagi 3-4 dosis

14
 Penatalaksanaan
Kortikosteroid
1. Prednison (Peroral) :
• Dapat diberikan bersamaan dengan makanan, dosis•
rendah <0,5 mg/kgBB/hari dibagi 3-4 dosis
• Dosis tinggi 1-2 mg/kgBB/hari (max. 60–80 mg/hari)
p.o, dibagi 3-4 dosis selama 3-6 minggu
2. Metil prednisolon (Parenteral)
• Dosis 30 mg/kg/hari i.v. (max. 1 g), selama 90 menit,•
3 hari berturut-turut, dilanjutkan tiap minggu,
disertai prednison dosis rendah setiap hari
3. Triamsinolon (Intraartikular)
Imunosupresan
• Azatioprin : Dosis anak 1-3 mg/kg/hari
Siklofosfamid : Diberikan secara per oral dengan
dosis 1–3 mg/kgBB/hari, parenteral Dosis awal 500 –
750 mg/m², max. 1 g/m²/hr bolus per infus, dosis
terendah dalam 150 ml larutan Dextrosa 5% selama
1 jam
Metotreksat : Dosis 7,5 mg peroral, 1x/minggu.
Bersama asam folat peroral
15
 Penatalaksanaan

Mikofenolat Mofetil (MMF)

• Merkaptopurin : Dosis 50-100 mg/hari
• Klorambusil : Dosis 0,1 mg/kgBB/hari
• Siklosporin A : Dosis anak belum diketahui. Dosis rendah dewasa: 3–6 mg/hari
Obat Topikal
• Betametason 0,05%
• Fluosinosid 0,05% untuk 2 minggu, selanjutnya diganti dengan Hidrokortison
Atasi Hipertensi
• Nifedipin dengan dosis 0,25-0,5 mg/kg/dosis dan dapat diulangi setiap 4-8
jam. Dosis per kali tidak lebih dari 10 mg
• Enalapril 2,5-5 mg/kg/hari diberikan dalam dosis tunggal
• Propranolol 0,5-1 mg/kg/hari dibagi dalam 2-4 dosis.
Suplementasi (Dosis kalsium karbonat (Caltrate) sebagai elemental kalsium)
• Usia < 6 bulan: 360 mg/hari
• Usia 6-12 bulan: 540 mg/hari
• Usia 1-10 tahun: 800 mg/hari
• Usia 11-18 tahun: 1.200 mg/hari
• Dosis Vitamin D (Hidroksikolekalsiferol): BB < 30 kg: 20 mcg per oral 3
kali/minggu, BB > 30 kg: 50 mcg per oral 3 kali/minggu
 Penatalaksanaan
Rehabilitasi Edukasi

• Olahraga diperlukan untuk• Istirahat

mempertahankan densitas tulang dan• Paparan sinar matahari
berat badan normal, olahraga yang
• Diet
dianjurkan adalah aerobik low impact
• Kelelahan dan tidur
• Alat khusus misalnya dapat dipakai
pelindung sendi
 Komplikasi
• Hepatitis lupus • Systemic Vasculitides

• Penyakit hati autoimun • Obat-obatan yang menyebabkan vasculitis

• Sirosis bilier primer • Sindrom antifosfolipid

• Cholangitis sclerosing primer • Koagulopati

• Hepatitis akibat virus • Trombositopenia

• Steatohepatitis • Kasus yang lebih jarang menyebabkan infeksi

• virus yang berat.

Fatty liver

• Kerusakan hati akibat obat

 nefritis, penyakit SSP, perdarahan paru, dan infark jantung. Infark
jantung disebabkan oleh pemakaian kortikosteroid kronis.

• Prognosis
Prognosis lupus bervariasi mulai dari ringan hingga berkembang
cepat menjadi berat disertai kegagalan multiorgan bahkan
kematian

• Pasien lupus tetap memiliki rerata mortalitas 2 -5 kali lipat lebih

tinggi daripada populasi umum.

• Prognosis pasien dengan lupus lebih buruk apabila disertai

dengan atau pada keadaan penyakit renal, hipertensi, usia muda,
ras kulit hitam, antibodi anti fosfolipid positif, aktivitas penyakit
yang tinggi.

• Penyebab mortalitas pada beberapa tahun pertama adalah

penyakit yang aktif (misal penyakit sistem saraf pusat atau ginjal)
atau infeksi yang berhubungan dengan imunosupresi.
 Kesimpulan
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VENUS JUPITER
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MARS SATURN
Despite being red, Mars Saturn is a gas giant
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 OUR PROCESS

MARS MERCURY
01 Despite being red, Mars
is a cold place
03 Mercury is the closest
planet to the Sun

VENUS
02 It’s terribly hot and it has
a beautiful name

25
 PATIENT CARE
Earth is the third planet from the Sun and the only one
that harbors life in the Solar System. This is where we all
live on
 PATIENT CARE

EARTH
It’s the planet
where we live

VENUS MARS
Venus has a PATIENT Mars is made of
beautiful name basalt

JUPITER
Jupiter is a gas
giant

27
AWESOME
WORDS
 AREAS WE COVER

JUPITER CERES VENUS MARS

AREA 1

AREA 2

AREA 3

AREA 4

29
 AREAS WE COVER
SURGERY CLINICS

MERCURY MARS JUPITER EARTH

Mercury is a Mars is made of Jupiter is a gas It’s the planet
small planet basalt giant where we live

● Subarea 1 ● Subarea 1 ● Subarea 1 ● Subarea 1

● Subarea 2 ● Subarea 2 ● Subarea 2 ● Subarea 2
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 AREAS DESCRIPTION

AREA 1 Mercury is a small planet

AREA 2 Saturn has several rings

AREA 3 Mars is made of basalt

AREA 4 Venus has a beautiful name

AREA 5 Jupiter is a gas giant

31
 SERVICES

MERCURY VENUS MARS

Mercury is the closest Venus is the second Despite being red, Mars
planet to the Sun and planet from the Sun. It’s is actually a cold place.
the smallest one in the hot and has a poisonous It’s full of iron oxide dust
Solar System atmosphere
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SATURN VENUS MARS

It’s composed It’s the second Despite being red,

ABOUT of hydrogen planet from the Mars is actually a
and helium Sun and is hot cold place

MED
STAFF 241 154 85

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 OUR LOCATIONS

MERCURY
Mercury is a
small planet

VENUS
Venus has a
beautiful name

MARS
Mars is made of
basalt

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 KEY ACCOMPLISHMENTS

MAIN GOALS IN 20X 20X 20 20X 20X 20X 20X

THE FUTURE X X XX X X X X
Venus is the second planet
from the Sun

Saturn has several and

beautiful rings

Despite being red, Mars is

actually a cold place
 95%
Mercury is the closest planet to the Sun and the
smallest one in the Solar System—it’s only a bit larger
than the Moon
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Mercury is the closest planet to the Sun and the
smallest one in the Solar System

VACCINES DIABETES CANCER

Venus has a beautiful Despite being red, Mars Jupiter is a gas giant
name, but also very high is actually a very cold and the biggest planet
temperatures place in the Solar System

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 INNOVATION STATUS

OUR ANNUAL PROGRESS

FROM 10% TO 60%
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 IMPROVEMENTS

VENUS MARS JUPITER

Venus has a beautiful It's full of iron oxide dust, Jupiter is a gas giant
name, but also very high which gives the planet and the biggest planet
temperatures its reddish cast in the Solar System

MERCURY SATURN NEPTUNE

Mercury is the closest Saturn is composed Neptune is the fourth-
planet to the Sun and mostly of hydrogen and largest planet in the
the smallest one helium Solar System
 IMPROVEMENTS

45% VENUS
Venus has a beautiful name, but
also very high temperatures

15% MARS
It's full of iron oxide dust, which
gives the planet its reddish cast

10% SATURN
Saturn is composed mostly of
hydrogen and helium

Follow the link in the graph to modify its

data and then paste the new one here.
30% NEPTUNE
Neptune is the fourth-largest
For more info, click here planet in the Solar System
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“Neptune is the farthest planet from “Saturn is a gas giant and has
the Sun and the fourth-largest by several rings. It's composed mostly
diameter” of hydrogen and helium”

JENNA DOE, 25 SUSAN BONES, 58

“Mercury is the closest planet to the “Earth is the third planet from the
Sun and the smallest in the entire Sun and the only one that harbors
Solar System” life in the Solar System”

JOHN JAMES, 36 TIMMY JIMMY, 47

 TESTIMONIALS
“Neptune is the farthest planet
from the Sun and the fourth-
largest by diameter”

JENNA DOE, 55

SUSAN BONES, 68
“Saturn is a gas giant. It's
composed mostly of hydrogen
and helium”
 TESTIMONIALS
“Mercury is the closest planet
to the Sun and the smallest in
the entire Solar System”

JOHN JAMES, 36

TIMMY JIMMY, 77
“Earth is the third planet from
the Sun and the only one that
harbors life”
 AWARDS

2000 2007 2013 2020

MERCURY VENUS MARS JUPITER

It’s the closest Venus has a Mars is actually a It’s the biggest
planet to the Sun beautiful name very cold place planet of them all

47
 AWARDS

20XX 20XX 20XX

VENUS MARS JUPITER
Venus has a beautiful It's full of iron oxide dust, Jupiter is a gas giant
name, but also very high which gives the planet and the biggest planet
temperatures its reddish cast in the Solar System

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