Kawasaki Disease

Darryl Shawn Godong

 Definisi
• Kawasaki disease (KD) merupakan suatu
vaskulitis dengan demam yang bersifat self
limiting, terutama menyerang anak dan balita.
• KD juga dikenal sebagai mucocutaneous lymph
node syndrome.
• Penyakit ini pertama kali ditemukan oleh
Tomisaku Kawasaki tahun 1967 di Jepang
Epidemiologi
Epidemiologi
 Epidemiologi
• Pada tahun 1980 mulai ditemukan efek jangka
panjang dari KD sebagai penyebab utama
acquired heart disease pada anak,
menggantikan penyakit jantung rematik
 Etiologi

IDIOPATIK

HIPOTESIS

SISTEM
GENETIK INFEKSI
IMUN
 Etiopatogenesis
Genetik
FAM167A-BLK on chromosome 8p23–p22
• B-lymphoid kinase (BLK )
• BLK is required for the development of interleukin (IL)-17-producing γδ T cells
• High levels of IL-17 and increased numbers of activated γδ T cells were reported
during the acute phase of KD

CD40, located on chromosome 20q12–q13.2

• Elevated CD40L expression during acute phase KD and significantly higher CD40L
expression in KD patients with coronary arterial lesions (CALs) were reported

Transforming growth factor (TGF)-b2, TGF-bR2 and SMAD3

• members of the TGF-b signalling pathway
• SNPs in these genes influ- ence KD susceptibility and coronary artery aneurysm
formation
 Etiopatogenesis
Genetik
FAM167A-BLK on chromosome 8p23–p22
• B-lymphoid kinase (BLK )
• BLK is required for the development of interleukin (IL)-17-producing γδ T cells
• High levels of IL-17 and increased numbers of activated γδ T cells were reported
during the acute phase of KD

CD40, located on chromosome 20q12–q13.2

• Elevated CD40L expression during acute phase KD and significantly higher CD40L
expression in KD patients with coronary arterial lesions (CALs) were reported

Transforming growth factor (TGF)-b2, TGF-bR2 and SMAD3

• members of the TGF-b signalling pathway
• SNPs in these genes influ- ence KD susceptibility and coronary artery aneurysm
formation
 Etiopatogenesis
Sistem Imun

•IL-1,IL-2, IL-6,IL-8, TNF-

Cytokines
∝

•monocyte chemotactic
Chemokines
protein-1 (MCP-1)
 Etiopatogenesis
Infeksi
Aktivasi sistem imun oleh agen infeksius terhadap host
dengan faktor genetik

Overlapping
Seasonal
clinical
clustering
features

Insiden yang
rendah pada
Epidemics
usia <3bulan
dan >4tahun
 Etiopatogenesis
• Peneliti menyimpulkan bahwa terjadi KD
disebabkan oleh siperantigen (molekul yang
meningkatkan sel T non spesifik,
mengakibatkan aktivasi sel T poliklonal dan
pelepasan citokin yang berlebih)
Patofisiologi
Gejala dan Tanda
Diagnosis
 Diagnosis Banding

Vaskulitis
• HSP

Viral dan Ricketsial

• Ehrlichiosis
• Rocky Mountain Spotted Fever
Rash
 Tatalaksana
• 2 g/kg dalam 10–12 jam pada 10 hari pertama
demam
IVIG • Jika demam dalam 48–72 jam setelah pemberian
pertama, pertimbangkan pemberian IVIG kedua

• 80–100 mg/kg/hari (4x/hari)

• dosis diturunkan menjadi 3-5mg/kg/hari (6-8
minggu atau kelainan jantung membaik)
Aspirin • setelah 48-72 jam bebas demam, atau
• 5 hari bebas demam, atau
• hari ke 14 demam

Kortikosteroid • methyl- prednisolone (30 mg/kg)

 Daftar Pustaka
• Hay W., Levin M. et al. Current Diagnosis and
Treatment. Ed 22. Mc Graw Hill Education. 2014.
• Schulman S., Rowley A. et al. Kawasaki disease: insights
into pathogenesis and approaches to treatment. 2015.
• Greco A., Virgilio A. et al. Kawasaki Disease: An
Evolving Paradigm. Autoimmunity Reviews. 2015.
• Advani N. Frekuensi Ekokardiografi pada Fase Awal
Penyakit Kawasaki. Departemen Ilmu Kesehatan Anak,
Fakultas Kedokteran Universitas Indonesia Rumah Sakit
Dr Cipto Mangunkusumo, Jakarta. 2018.
• Kasper, Fauci, Hauser. Harrison’s Principles of Internal
Medicine. 19ty Ed. Mc Graw Hill Education. 2015.