Kawasaki Disease 6sa2

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 GENETIK

SISTEM IMUN

INFEKSI

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

Etiopatogenesis Sistem Imun

Cytokines

•IL-1,IL-2, IL-6,IL-8, TNF∝

•monocyte chemotactic Chemokines protein-1 (M-1)

Etiopatogenesis Infeksi Aktivasi sistem imun oleh agen infeksius terhadap host dengan faktor genetik

Overlapping clinical features

Seasonal clustering

Epidemics

Insiden yang rendah pada 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 IVIG

Aspirin

Kortikosteroid

• 2 g/kg dalam 10–12 jam pada 10 hari pertama demam • 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) • setelah 48-72 jam bebas demam, atau • 5 hari bebas demam, atau • hari ke 14 demam

• 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, Ha. Harrison’s Principles of Internal Medicine. 19ty Ed. Mc Graw Hill Education. 2015.