Kawasaki DiseaseEmily Caudle

Jill CollinsMaria Cangiani

AKA..◦ mucocutaneous lymph node syndrome

-OR-◦ infantile polyarteritis

Characterized by an acute generalized systemic vasculitis occurring throughout the body

Self-limiting and is the most common cause of acquired heart disease in children in Japan and the U.S.

Over 80% of patients with Kawasaki disease are 4 years of age or younger◦ Male to female ratio of 4:1

Kawasaki Disease

Etiology remains unknown◦ Clinical evidence supports an infectious cause

Most exclusively affects young children ◦ with peak incidence between 13 and 24 months◦ Increased rate of spread among children are low

Exhibits geographic and seasonal outbreaks◦ Late winter and early spring

Between 9.1 and 32.5 per 100,000 children contract the disease each year in the U.S.◦ Incidence is HIGHER in Asian-American children, followed by African

Americans, Hispanics and LOWEST in White children

Epidemiology

Pathophysiology Disease is characterized by VASCULITIS ◦ begins in the small vessels and progresses to involve some of

the larger arteries Immunologic abnormalities ◦ increased activation of helper T cells◦ increased levels of immune mediators and antibodies

destroy endothelial cells have been detected during the acute phase of the disease

Pathophysiology

◦ Stage 1: acute phase (0-14 days) begins with abrupt onset of high fever that is unresponsive to antipyretics or antibiotics Significant irritability bilateral nonpurulent conjunctival

injection, erythema or the oropharynx dryness and fissuring of the lips “strawberry tongue” cervical lymphadenopathy polymorphous rash erythema of urethral meatus tachycardia edema of extremities

Lab findings: an elevated ESR platelet count positive CRP leukocytosis with left shift

slight decreases in RBCs and hemoglobin.

Initially platelets may be normal with gradual increase after 7th day of fever

Clinical Manifestations: 4 stages

Stage 2: subacute phase (2-4 weeks after onset)◦Begins with resolution of fever and lasts until all other

clinical signs have disappeared◦Desquamation of the fingers occurs first, followed by toes◦Coronary artery aneurysms appear during this period in

15% -25% of untreated children and less than 5% of treated children

◦Death from the disease occur from cardiac sequelae 15-45 days after onset of fever

Clinical Manifestations

Stage 3: convalescent phase◦All clinical signs have resolved◦ Lab values may not have returned to normal◦Phase complete when all blood values normal

6-8 weeks from onset Stage 4: chronic phase◦ 40 days to years after illness◦Coronary complications, if present, can persist into

adulthood◦Children with coronary dilation or aneurysms may have

long-term coronary endothelial changes, which place the child at risk for early ischemic disease

Clinical Manifestations

Child must exhibit FEVER for 5 days plus four of the other five criteria, or, if fewer than four criteria, coronary vessel involvement:◦Bilateral conjunctival injection without exudate◦Polymorphous rash that may be uticarial or pruritic◦ Inflammatory changes in the lips and oral cavity◦Changes in the extremities, such as peripheral edema,

erythema of the palms and soles, or desquamation of the hands and feet

◦Cervical lymphadenopathy that is often unilateral, anterior cervical

Diagnostic Criteria

Diagnostic Criteria (images)

Conjuctival erythema

BILATERAL CONJUNCTIVAL INJECTION

Oral mucous membrane changes,

injected or fissured lips,

injected pharynx

Diagnostic continued…

The word “injected” means RED

Strawberry Tongue

Diagnostic continued…..

PERIUNGUAL DESQUAMATION (Convalescent phase)

Peripheral extremity changes, including erythema of palms or soles, edema of hands or feet (acute phase), and periungual desquamation (convalescent phase)

ERYTHEMA of palms/soles

(Acute phase)

The diagnostic of Kawasaki disease is based on clinical manifestations. The CDC requires that fever and four of the six other criteria listed above in stage I be demonstrated.

Electrocardiogram, echocardiogram, cardiac catheterization, and angiocarddiography may be required to diagnose cardiac abnormalities.

Diagnostic evaluation

Although there are no specific laboratory tests, the following may help support diagnosis or rule out other disease.◦ 1. CBC

Leukocytosis during acute stage.◦ 2. Erythrocytes and hemoglobin

slight decrease.◦ 3. Platelet count

increased during second to fourth week of illness.◦ 4. IgM, IgA, IgG, and IgF

transiently elevated.◦ 5. Urine

protein and leukocytes present.◦ 6. Acute phase reactants

(ESR, C-reactive protein, alpha I antitrypsin) are elevated during the acute phase.

◦ 7. Myocardial enzyme levels (serum CK-MB) suggest MI if elevated.

◦ 8. Liver enzymes (AST, ALT) moderately elevated.

◦ 9. Lipid profile low high density lipoprotein and high triglyceride level.

Differential Diagnoses

Kawasaki Disease

Measles

Adenovirus

Scarlet Fever

Drug reaction

Stevens-Johnson

Syndrome

Erythema Multiforme

Kawaskaki Disease

Leptospirosis

Inflammatory Bowel

Disease

SLE

Sarcoidosis

Rickettsial Infection

Toxic shock

WHAT ELSE COULD IT BE ??

Kawasaki disease has replaced acute rheumatic fever as the most common cause of acquired heart disease in children.

Rheumatic fever (RF) is an inflammatory disease that may develop after an infection with Streptococcus bacteria (such as strep throat or scarlet fever). The disease can affect the heart, joints, skin, and brain. ◦ It arises as a complication of untreated or inadequately

treated strep throat infection. Rheumatic fever can seriously damage the valves of the heart.

Kawasaki VS.

Acute Rheumatic Fever

Abdominal pain Fever Heart (cardiac) problems◦ which may not have symptoms, or may result in shortness of breath and

chest pain Joint pain, arthritis ◦ mainly in the knees, elbows, ankles, and wrists

Joint swelling; redness or warmth Nosebleeds (epistaxis) Skin nodules Skin rash (erythema marginatum) ◦ Skin eruption on the trunk and upper part of the arms or legs◦ Eruptions that look ring-shaped or snake-like

Sydenham chorea ◦ emotional instability, muscle weakness and quick, uncoordinated jerky

movements that mainly affect the face, feet, and hands

Symptoms of RF

Contrasting KD and RF

Kawasaki DiseaseAcute Rheumatic Fever

Once diagnosed…◦ immediate treatment

should be started

Manangement plan for KD

The recommended initial therapy includes:◦ IVIG

(2 gm/kg) administered as a single infusion over 8 to 12 hours

◦Aspirin (initial dose of 80 to 100 mg/kg daily divided into four doses).

 ◦ The AHA and the AAP recommend these two

medications for the treatment of acute KD.

(Additional agents are used only for children who fail to respond to standard therapy)

1st Line of treatment

CARDIOVASCULAR The most common and potentially life-threatening complication of KD

is coronary artery aneurysm. The aneurysm is a result of the chronic inflammation of the blood

vessels (vasculitis) which causes a weakening in the vessel wall.◦ The aneurysm can eventually burst leading to internal bleeding or more

often, blood clots form in the area leading to occlusion of the coronary artery and myocardial infarction.

If untreated, up to 25% of patients with KD develop coronary artery aneurysms. Most aneurysms develop within 6-8 weeks from the onset of illness. If treatment is started within 10 days of the diagnosis, the incidence of coronary artery disease/complications drops to approximately 2%.

Complications of Kawasaki Disease

CARDIOVASCULAR Other possible cardiac complications include:

MyocarditisPericarditisCHFPericardial effusionMitral insufficiencyAortic insufficiencyArrythmias

Complications

LONG TERM CARDIAC SEQUELAE A multi-centre follow-up study was done in Japan

obtaining cardiac status on 1594 patients who presented with KD in 1996. Of the 1338 in whom follow up data was available, 10.3% had cardiac sequelae at 1 month and 4.2% at 1 year. The prevalance was greater in males. About 50% of aneurysms regressed within 5 years. The main cause of death in KD is myocardial infarction secondary to thrombosis of an aneurysm or stenosis.

Complications

GASTROINTESTINAL

DiarrheaVomitingAbdominal painHydrops of the gallbladderElevated liver enzymesHepatomegalyAcute surgical abdomen

Complications

NEUROLOGICAL

IrritabilityAseptic meningitisTransient hemiplegiaCerebral infarctionAtaxiaSeizuresFocal encephalopathyLethargyFacial palsy

Complications

RENAL

ProteinuriaHematuriaSterile pyuriaEchogenic kidneysRenal failure (rare)

Complications

HEMATOLOGICAL

Haemophagocytic syndrome (AKA…Hemophagocytic lymphohistiocytosis (HLH))-a rare condition caused by excess activation and proliferation of macrophages.

Complications

At this point in time, the exact cause of Kawasaki Disease remains unknown and so it is unknown how to prevent the onset of the disease.

Some believe it is caused by a virus or bacteria but this theory has never been proven.

It is important, however, to note that when the disease is diagnosed and treatment is initiated in the very early stages, all most all of the complications that were discussed previously can be prevented. Full recovery can be expected for most patients diagnosed with the disease.

Prevention Measures

Patient education about this disease revolves around early recognition of the symptoms as well as seeking treatment as soon as possible. There is a great educational handout on Kawasaki Disease that can be obtained by visiting the American Family Physician website at http://www.aafp.org/afp/990600ap/990600c.html. The handout is copyrighted but permission is given to print and photocopy for nonprofit educational uses.

Patient Education

Just a couple tidbits of information found on Kawasaki Disease that may be of interest to some:

John Travolta’s son, Jett was diagnosed with Kawasaki Disease at the age of two.

There has been some speculation that the vaccine used to help prevent rotavirus infection (RotaTeq) has been linked to development of Kawasaki Disease. ◦ To this date there has not been enough data to support this claim and the

CDC continues to support the safety and effectiveness of the RotaTeq vaccine in preventing rotavirus infection. For more information on this visit http://www.cdc.gov/vaccinesafety/vaccines/rotavirus.html.

FYI

http://www.youtube.com/watch?v=thdcueIequ0&feature=player_detailpage

ENJOY and THANK YOU!

Video of Kawasaki Disease

Bulbar conjunctiva (2011). Retrieved from http://www.britannica.com/EBchecked/topic/84026/bulbar-

conjunctiva

Burns, C., Dunn, A., Brady, M., Starr, N., & Blosser, C. (2009). Cardiovascular disorders. In S. Clark

(Ed.), Pediatric Primary Care (pp. 758-764). St. Louis, MO: Saunders Elsevier.

Celebrity sentry. (n.d.). Retrieved June 30, 2011, from http://www.celebritysentry.com/post/kawasaki-

disease/

Chin, T. K., & Jung, L. K. (2010, February 25). Pediatric Rheumatic Fever. Medscape. Retrieved from

http://emedicine.medscape.com/article/1007946-overview#a0101

Fisman, D. N. (2000, Nov-Dec). Hemophagocytic syndromes and infection. Emerging Infectious

Diseases, 6(6). Retrieved from http://www.cdc.gov/ncidod/eid/vol6no6/fisman.htm

Jatla, K. K. (2011). Medscape reference. In H. Roy, Sr (Ed.), Ophthalmologic Manifestions of Kawasaki

Disease. Retrieved from http://emedicine.medscape.com/article/1197545-overview#aw2aab6b3

KD Foundation. (2010, September 8). Kawasaki Disease [Video file]. Retrieved from

http://www.youtube.com/watch?v=thdcueIequ0&feature=player_detailpage   

References

Kawasaki disease. (1999, June). American Academy of Family Physicians. Retrieved from

http://www.aafp.org/afp/990600ap/990600c.html

Kawasaki syndrome and RotaTeq vaccine. (2011, February 8). Centers for Disease Control and Prevention:

Vaccine Safety. Retrieved from http://www.cdc.gov/vaccinesafety/vaccines/rotavirus.html

Ogershok, P. R. (2009, August 6). Kawasaki Disease. Medscape Reference. Retrieved from

http://emedicine.medscape.com/article/330081-overview

Porth, C. M., & Matfin, G. (2008). Pathophysiology: Concepts of altered health states (8th ed.). Philadelphia,

PA: Lippincott Williams & Wilkins.

Sundel, R. (2011, January). Epidemiology and etiology of Kawasaki disease. UpToDate. Retrieved from

http://0-www.uptodate.com.topekalibraries.info/contents/epidemiology-and-etiology-of-kawasaki-disease?

source=search_result&selectedTitle=3%7E150

Tizard, E. J. (2005). Complications of Kawasaki disease. Current Pediatrics, 62-68.

References