kawasaki disease

Prof DR Dr Arijanto Harsono SpAK

1967 Kawasaki Tomisaku reported 50 cases with febrile,

servical lymphadenopathy, conjunctival redness,

red tongue, cracked lips, erythema, and swollen hands and feet exfoliation followed

PENDAHULUAN

KAWASAKI DISEASE (KD)Previously called: MUCOCUTANEOUS LYMPH NODE SYNDROMEVasculitis DISEASES AFTER THE SECOND MOST HENOCH Schönlein Purpura

2Prof DR Dr Ariyanto Harsono SpA(K)

ETIOLOGY/PATHOGENESIS

Coronary vasculitis and medium blood vessels

Super-Antigen exposure HSP65

Activation of the immune system

Activation of endothelial cells and monocytes

Activation of T cells and B cells

Increased inflammatory cytokines


Prof DR Dr Ariyanto Harsono SpA(K)

ETIOLOGY/PATHOGENESIS...


Clinical Manifestations


Clinical Manifestations...


ClNICAL MANIFESTATION...


CLINICAL MANIFESTATION...


CLINICAL MANIFESTATIONS...



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CLINICAL MANIFESTATIONS


CLINICAL MANIFESTATIONS……

CLINICAL MANIFESTATIONS …


http://content.nejm.org/content/vol356/issue7/images/large/03f1.jpeg


CLINICAL MANIFESTATIONS……..


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CLINICAL MANIFESTATIONS……..

CLINICAL MANIFESTATIONS…….

MANIFESTASI KLINIS...



Unusual manifestation

Prof DR Dr Ariyanto Harsono SpA(K) 26

Angus’ Rash

Unusual manifestation…..





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Beau’s line

DIAGNOSISDiagnosis Criteria:

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Bilateral non-purulent conjunctivitis

Changes in lips and oral cavity

Peripheral limb abnormalities

Polymorph EksantemCervical adenopathy

Typical KD: Fever 5 days with 4 or more of the following criteria

DIAGNOSISKriteria Diagnosis:

Typical KD: Demam 5 hari atau lebih disertai 4 dari kriteria dibawah ini

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PolymorphEksantemCervical adenopathy



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Polymorph ExanthemCervical adenopathy



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Polymorph Eksanthem

Cervical adenopathy



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Polymorph Eksanthem

Cervical adenopathy


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Polymorph Eksanthem

Cervical adenopathy

DIAGNOSISDiagnosisCriteria:

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Polymorph Eksanthem

Cervical adenopathy

DIAGNOSISDiagnosis Ceriteria:

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Eksantem polymorphCervical adenopathy

Atypical KD:

Fever 5 days or more criteria +3 or lessFever 5 + days or more coronary abnormalities


• No history of sore throat or evidence of tonsillar exudate

• Streptococcal serology: negative.

.

• Scarlet fever is thought to be unlikely.


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Laboratry Examinations

Prof DR Dr Ariyanto Harsono SpA(K) 44

LED

CRP

Leukocyte

Neutrophil

Thrombocyte

CholesterolHDLTrigliserid

Plasma Cell IgAPerinuclear anti-neutrophilic cytoplasmic antibodies (P-ANCA): +

Pathology examination...

Intima and surrounding tissue necrosis. Areas of necrosisshowed fibrinoid change and basophilik. Inflammatory cell infiltrates-2 and the rest of the core seen in areas of necrosis.

Picture of early necrosis; smooth muscle showed cytoplasmic acidofiliaCore looks picnotic (chromatin condensation).Adventisia contained infiltrates of inflammatory cells.


Arteriole: fibrinoid necrosis (leukocytoclastic vasculitis): Note the pink staining material (fibrinoid necrosis) in multifocal areas of the

thickened wall of the venule. The material represents protein derived from the plasma that has deposited in the vessel wall owing to an increase in vessel

permeability from the inflammatory process.

• It is called fibrinoid because it looks like fibrin in a clot but it is really protein. Small vessel vasculitis is usually due to immune complex (IC) disease (type III hypersensitivity). ICs are deposited in the vessel wall and then activate the complement system. C5a, a chemotactic factor, attracts neutrophils (only a few are visible at around 7 o’clock).


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MANAGEMNT


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IVIG: Should be given within the

first 10 days after the diagnosis is established

Dosage: 2g/Kg single dose Children: 1g/kg BW should

be given "Single infusion" in 8-12 hours. If you already have heart problems given in divided doses 3-4 days


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Management…

Patients who are refractory to IVIG: Pulse methyl prednisolone 0.5-2 mg / kg bw

Aspirindose:50-80 mg / kg bw in the acute inflammatory phase3-5 mg / kg bw after fever resolved and platelets increased, maintained until cardiac abnormalities improved

Dipridamole: In patients who are intolerant to aspirinDose: 2-3 mg / kg bw

50Prof DR Dr Ariyanto Harsono

SpA(K)


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PROGNOSIS

Were largely complete recoveryLong-term observation of patients with aneurysms remaining 50% improved cardiac abnormalitiesMortality 1970: 1-2%; 1990: 0.4% due to the blockage of coronary / other cardiac abnormalities95% of deaths occurred after 6 months because of infection, the rest after 10 years


ProfilaksisDentists should remain alert

to features of the acute disease, and in patients with a history of Kawasaki disease, be aware of the possibility of recurrence and of heart valve defects requiring antibiotic prophylaxis prior to relevant dental treatment.Prof DR Dr Ariyanto Harsono SpA(K) 53

Prof Kawasaki Karlee

54)


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Prof Takashimura

kawasaki disease

Health & Medicine

diagnosiskriteria diagnosis

hari ataulebih disertai

cracked lips

atypical kd

diagnosisdiagnosis criteria

diagnosisdiagnosis ceriteria

monocytesactivation

servical lymphadenopathy