Kawasaki Disease (KD)

• Or mucocutaneous lymph node syndrome• Described first by Tomisaku Kawasaki in Japan,

1967.• An acute inflammatory syndrome that takes the

form of systemic vasculitis, and predominantly affects children.

• Important complications of this disease are coronary artery dilation and aneurysm formation.

• 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

• Coronary artery aneurysms or ectasia: 15-25% of those untreated– Myocardial infarction, sudden death, ischemic cardiac disease

Illness can’t be explained by other disease

Kawasaki Disease: Classic Characteristics

clinical features of Kawasaki disease

Peeling and erythema of the fingertips.

Strawberry tongue.

http://emedicine.medscape.com/article/804960-overview

Pathophysiology• The etiology of Kawasaki disease remains

unknown, although many suspect an infectious etiology.

• the self-limited nature; the winter and summer presentations; and the characteristic fever, adenopathy, and eye signs.

• many factors (viruses, staphylococci "super antigens") : triggering a final common pathway that results in immune activation.

• May be related to a genetic predisposition to genetic polymorphisms.

Causes

• Multiple theories exist :– An infectious etiology.– an immunological abnormality.– Clinical and epidemiologic features support an

infectious etiology, but many authorities believe that an autoimmune component also exists.

Pathology

• The media of affected vessels demonstrate edematous dissociation of the smooth muscle cells.

• Endothelial cell swelling & subendothelial edema are seen (but the internal elastic lamina remains intact).

• An influx of neutrophils is found in the early stages (7 to 9 days after onset) , also Lymphocytes and IgA plasma cells.

Pathology

• Destruction of the internal elastic lamina and eventually fibroblastic proliferation.

• Matrix metallo proteinases (MMP) are prominent in the remodeling process.

• Active inflammation is replaced over several weeks

to months by progressive fibrosis, with scar formation.

• Active remodeling with intimal proliferation and neoangiogenesis.

Pathogenesis

http://www.nature.com/nrmicro/journal/v6/n5/fig_tab/nrmicro1853_F1.html#figure-title

Pathogenesis

http://www.nature.com/nrmicro/journal/v6/n5/fig_tab/nrmicro1853_F1.html#figure-title

Pathogenesis

Proposed role of S100 proteins (myeloid-related protein [MRP]-8, MRP-14, and S100A12) in the genesis and maintenance of the vasculitis in Kawasaki disease.

Pathogenesis

The net result of S100 protein binding is platelet aggregation and adherence to endothelium.

Complications

Symptoms often disappear within just 2 days of the start of treatment. ภาวะแทรกซ้�อนท��อาจเก�ดขึ้��นในกรณี�ท��ไม่�ได�ร�บการร�กษา ม่�โอกาสเก�ด coronary artery aneurysm ได�ร�อยละ 20Serious complications :

vasculitis affect the coronary arteries, which supply blood to the

heart.

Arrhythmias or abnormal functioning of some heart valves also can occur.

Complications

Depressed myocardial contractility . Giant coronary artery aneurysms. Peripheral arterial obstruction . Serum lipid abnormalities. Renal disease and urinary abnormalities. Noncoronary vascular involvement. Macrophage activation syndrome.

Treatment

Treatment should begin as soon as possible, ideally within 10 days of when the fever begins. Admit to monitor cardiac function

Complete cardiac evaluation – CXR, EKG, echo

gamma globulin (purified antibodies), an ingredient of blood that helps the body fight infection.The child also might be given a high dose of aspirin to reduce the risk of heart problems.

Treatment

• Aspirin– High dose initially: 80 – 100 mg/kg/day in 4 doses– Low dose: 3 – 5 mg/kg/day

• IVIG– 2 g/kg as single infusion ( within 10 -12 hr.)

Treat within 10 days of onset of symptoms

Risks of Treatment

• IVIG– Flushing, hypotension, chills, fever, headache, low

back pain, nausea, chest tightness– Rare: aseptic meningitis, neutropenia, elevated creatinine, hemolytic anemia– IgA deficient patients at risk for anaphylaxis– Risks of blood product

• Aspirin – Concern for Reye syndrome

References• http://kidshealth.org/parent/medical/heart/kawasaki.html.• http://emedicine.medscape.com/article/804960-overview• http://www.nature.com/nrmicro/journal/v6/n5/fig_tab/

nrmicro1853_F1.html#figure-title• http://www.heart.org/HEARTORG/Conditions/Kawasaki-

Disease_UCM_308777_Article.jsp• http://content.onlinejacc.org/cgi/content/full/48/6/1265/FIG1• http://www.ra.mahidol.ac.th/dpt/PD/knowdocth/Kawasaki%20Disease• http://www.ncbi.nlm.nih.gov/pubmed/16990356• http://www.med.cmu.ac.th/dept/pediatrics/06-interest-cases/ic-79/Ped401-

Exanthemotous%20Fever-thanyawee.pdf