kawasaki disease

KAWASAKI DISEASE Dr. Sachin Soni

DNB Pediatrics

Indraprastha Apollo Hospital,

New Delhi

www.dnbpediatrics.com


What is it?

Medium vessel vasculitis presents as acute febrile illness of childhood, characterized by coronary arteries anomaly in 15-25% of affected individuals


Epidemiology Its common pediatric disorder with the annual

incidence range 60-150 per 100,000

Commonest pediatric vasculitis in children below 5 years of age(1-3)

Commonest vasculitic disorder amongst all ages


Etiology Genetics

Autoantigen

Toxin mediated (STSS) super antigen mediated


Pathogenesis

Arterial stenosis or occlusion

vascular wall can become progressively fibrotic, marked intimal proliferation

Thrombi in lumen and obstruct blood flow

Dilation (ectasia) saccular or fusiform aneurysm formation

Loss of structural integrity weakens the vessel wall

Destruction of the internal elastic lamina

In most severely affected vessels, inflammation involves all three layers of the vascular wall

IgA plasma cells are prominent in the inflammatory infiltrate

Eedema of endothelial and smooth muscle cells

Acute or subacute stages: Inflammatory infiltration of vascular wall, initially polymorphonuclear cells thereafter by macrophages, lymphocytes

(primarily CD8+ T cells), and plasma cells


EPIDEMIOLOGIC CASE DEFINITION (CLASSIC CLINICAL CRITERIA)

Fever persisting for at least 5 days Presence of at least 4 principle feature

1- Changes in extremities:-

A- Acute:- Erythema of palm, soles and edema of hand and feet

B- Sub acute:- Periungul peeling of finger, toes in 2-3 polymorphus exanthema

2- B/L Bulbar conjunctival injection without exudates

3- Changes in lip and oral cavity:- Erythema, lip cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosa

4- Cervical lymphadenopathy (>1.5 cm diameter), usually unilateral


Other Clinical Findings

Cardiovascular findings Congestive heart failure, myocarditis, pericarditis, valvular regurgitation,

Coronary artery abnormalities, Aneurysms of medium-sized noncoronary arteries

Raynaud phenomenon, Peripheral gangrene

Musculoskeletal system Arthritis, arthralgias

Gastrointestinal tract Diarrhea, vomiting, abdominal pain, Hepatic dysfunction, Hydrops of gallbladder

Central nervous system Extreme irritability, Sensoryneural hearing loss, Aseptic meningitis,

Genitourinary system Urethritis/meatitis


Clinical Menifestation


Percentages of cases

CAD

Diarrhea

LAP

BCG

pyuria

induration

polymorphus skin rashes

desquamation

conjunctivitis

straberry tongue

Fever

0 10 20 30 40 50 60 70 80 90 100


Laboratory finndings

Complete Hemogramme AnemiaLeukocytosis with neutrophilia and immature formsThrombocytosis after one week Elevated erythrocyte sedimentation rate (ESR)

Acute phase reactant Elevated CRP

LFTs Hypoalbuminemia, Elevated serum transaminases, Elevated serum gamma glutamyl transpeptidase, Abnormal plasma lipids

CSF CSF Pleocytosis

others Hyponatremia, Leukocytosis in synovial fluid, sterile pyuria


Strawberry tongue


Congestion of bulbar conjunctiva


Indurative edema of the hands


Desquamation of the fingers


Coronary Artery Aneurysm

Coronary angiogram demonstrating giant aneurysm of the left anterior descending coronary artery (LAD) with obstruction and giant aneurysm of the right coronary artery (RCA) with an area of severe narrowing in 6 yr old boy


Two-dimensional echocardiography

Most useful test to monitor development of coronary artery abnormalities

Brightness of the arterial walls and lack of normal tapering of the vessels

Coronary artery dimensions, adjusted for body surface area (BSA), are significantly increased in the first 5 wk after presentation

BSA-adjusted coronary artery dimensions on baseline echocardiography in the first 10 days of illness to be good predictors of CAD


Aneurysm defined by Japanese Ministry of Health classified as:-

Small (<5 mm internal diameter) Medium (5-8 mm internal diameter) Giant (>8 mm internal diameter)

Echocardiography performed at diagnosis and after 2-3 wk of illness

If the results are normal, repeat study should be performed 6-8 wk after onset of illness


Diagnosis

Classic KD:- Diagnostic criteria- Fever for at least 4 days and at least four of five of

above principal characteristics of the illness

Atypical or incomplete KD:- Patients have persistent fever but fewer than four of the

five characteristics In these patients, laboratory and echocardiographic data

can assist in the diagnosis Incomplete cases are most frequent in infants, who,

unfortunately, also have the highest likelihood of development of coronary artery abnormalities


Differential Diagnosis

VIRAL INFECTIONS Adenovirus,Enterovirus,Measles,EBV

BACTERIAL INFECTIONS

Scarlet fever, Rocky Mountain spotted fever, Leptospirosis, Bacterial cervical lymphadenitis

RHEUMATOLOGIC DISEASE

Systemic-onset juvenile idiopathic arthritis

OTHER Toxic shock syndrome, Staphylococcal scalded skin syndrome, Drug hypersensitivity reactions, Stevens-Johnson syndrome


TREATMENT

Acute phase:-

Intravenous immunoglobulin 2 g/kg over 10-12 hr

AND

Aspirin 80-100 mg/kg/day divided every 6 hr orally

until patient is afebrile for at least 48 hr


CONVALESCENT STAGE

Aspirin 3-5 mg/kg once daily orally until 6-8 wk after illness onset


LONG-TERM THERAPY FOR PATIENTS WITH CORONARY ABNORMALITIES

Aspirin 3-5 mg/kg once daily orally

Clopidogrel 1 mg/kg/day (max 75 mg/day)

Most experts add warfarin or low-molecular-weight heparin for those patients at particularly high risk of thrombosis


ACUTE CORONARY THROMBOSIS

Fibrinolytic therapy with tissue plasminogen activator

Or

Other thrombolytic agent under supervision of a pediatric cardiologist


Complication

Coronary artery aneurysm Acute thrombosis Coronary artery stenosis Reyes syndrome


IVIG-RESISTANT KD

• Defined by persistent or recrudescent fever 36 hr after completion of the initial IVIG infusion• Another dose of IVIG at 2 g/kg is administered to patients with IVIG resistance.• Intravenous methylprednisolone

• If a second dose of IVIG or corticosteroids are ineffective• Cyclophosphamide and plasmapheresis.• Tumor necrosis factor inhibitor infliximab

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kawasaki disease

Health & Medicine

right coronary artery

coronary arteries anomaly

giant aneurysm

t cells

polymorphonuclear cells

days of illness

wk of illness

vascular wall destruction