Prof. Dr. Saad S Al AniSenior Pediatric Consultant

Head of Pediatric DepartmentKhorfakkan Hospital ,

Sharjah, UAEsaadsalani@yahoo.com

Introduction

• Kawasaki disease, formerly known as mucocutaneous lymph node syndrome or infantile polyarteritis nodosa, is an acute febrile vasculitis of childhood first described by Dr. Tomisaku Kawasaki in Japan in 1967.

Idiopathic multisystem disease characterized by vasculitis of small & medium blood vessels, including coronary arteries

Definition

Cont.

• Worldwide, with Asians at highest risk.

• Approximately 20% of untreated patients develop coronary artery abnormalities

• Leading cause of acquired heart disease in children in the United States and Japan.

Etiology

• The cause of the illness remains unknown, but clinical and epidemiologic features strongly support an infectious origin.

• ? genetically predisposed hosts

Epidemiology

• 3,000 cases are diagnosed annually in the United States.

• The incidence in Asian children is substantially higher than in other racial groups, .

• In Japan, more than 170,000 cases have been reported since the 1960s.

Cont.

• The illness occurs predominantly in young children;

• 80% of patients are younger than 5 yr

• only occasionally are teenagers and adults affected.

Pathogenesis

Severevasculitis

Edema of endothelial

and smooth muscle cells

Intense inflammatory infiltration of

the vascular wall

Weakens, resulting in dilatation or

aneurysm formation

Thrombi

Fibrosis , intimal proliferation,

stenosis

Pathogenesis (cont.)

An inflammatory infiltrate in *myocardium

*upper respiratory tract *pancreas

*Kidney * biliary tract

Clinical Manifestations

• Kawasaki disease is generally divided into three clinical phases:

1.The acute febrile phase

2. The subacute phase

3. The convalescent phase

1.The acute febrile phase - which usually lasts 1-2 wk - is characterized by fever and the other acute signs of illness.

2. The subacute phase

- Begins when fever and other acute signs have abated, but irritability, anorexia, and conjunctival injection may persist.

- is associated with: * desquamation * thrombocytosis * development of coronary aneurysms * highest risk of sudden death. - This phase generally lasts until about the 4th

wk.

3.The convalescent phase

-begins when all clinical signs of illness have disappeared

- continues until the erythrocyte sedimentation rate (ESR) returns to normal, approximately 6-8 wk after the onset of illness.

Diagnostic Criteria

- Fever for 5 or more days- Presence of 4 of the following:1. Bilateral conjunctival injection2. Changes in the oropharyngeal mucous

membranes3. Changes of the peripheral extremities 4. Rash 5. Cervical adenopathy- Illness can’t be explained by other disease

Lab Features

WBC ESR, positive CRP

• Anemia

• Mild transaminases albumin

• Sterile pyuria, aseptic meningitis platelets by day 10-14

Differential Diagnosis

• Measles• Scarlet fever• Drug reactions• Viral exanthems• Toxic Shock Syndrome• Stevens-Johnson Syndrome• Systemic Onset Juvenile Rheumatoid Arthritis• Staph scalded skin syndrome

Intravenous immunoglobulin 2 g/kg over 10-12 hr with aspirin 80-100 mg/kg/24 hr divided every 6 hr orally until 14th illness day

1.Acute stage

Treatment

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

2.Convalescent stage

3.Long-term therapy for those with coronary abnormalitiesAspirin 3-5 mg/kg once daily orally ±

dipyridamole 4-6 mg/kg/24 hr divided in two or three doses orally (most experts add warfarin for those patients at particularly high risk of thrombosis)

4.Acute coronary thrombosis

Prompt fibrinolytic therapy with tissue plasminogen activator, streptokinase, or urokinase under supervision of a pediatric cardiologist

Complications

• Recovery is complete and without apparent long-term effects for patients who do not develop coronary disease

• Recurrent illness occurs in only 1-3% of cases

Prognosis• The prognosis for patients with coronary

abnormalities depends on the severity of coronary disease. ,In Japan, fatality rates are now less than 0.1%.

• 50% of coronary artery aneurysms resolve echocardiographically by 1-2 yr after the illness

References• Akagi T, Ogawa S, Ino T, et al: Catheter interventional treatment in Kawasaki

disease: A report from the Japanese Pediatric Interventional Cardiology Investigation Group. J Pediatr 2000;137:181-6. Medline Similar articles

• American Heart Association Council on Cardiovascular Disease in the Young, Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease: Diagnostic guidelines for Kawasaki disease. Circulation 2001;103:335-6.

• Brown TJ, Crawford SE, Cornwall M, et al: CD8 T cells and macrophages infiltrate coronary artery aneurysms in acute Kawasaki disease. J Infect Dis 2001;184:940-3. Medline Similar articles

• Chang RKR: Hospitalizations for Kawasaki disease among children in the United States, 1988-1997. Pediatrics 2002;109:e87.

• Han RK, Silverman ED, Newman A, et al: Management and outcome of persistent or recurrent fever after initial intravenous gammaglobulin therapy in acute Kawasaki disease. Arch Pediatr Adolesc Med 2000;154:694-9. Medline Similar articles

• Rowley AH, Shulman ST, Mask CA, et al: IgA plasma cell infiltration of proximal respiratory tract, pancreas, kidney, and coronary artery in acute Kawasaki disease. J Infect Dis 2000;182:1183-91. Medline Similar articles

• Stockheim JA, Innocentini N, Shulman ST: Kawasaki disease in older children and adolescents. J Pediatr 2000;137:250-2. Medline Similar articles