Kawasaki disease – what is new?

Dr. Surjit Singh

MD; DCH (Lon.); FRCP (Lon.); FRCPCH (Lon.); FAMS

Professor of Pediatrics, Allergy and Immunology Unit,Advanced Pediatrics Center,

PGIMER, Chandigarh&

Principal Investigator, Indian Council of Medical Research Centre for Advanced Research in Primary Immunodeficiency Diseases

What is new in epidemiology of Kawasaki disease?

Epidemiology of KD

Arch. Dis. Child 2015

Kawasaki disease: circa 2016

• Kawasaki disease is the commonest vasculitic disorder in children

• KD is several times more common than all the other vasculitic disorders combined

• 1% of all children born in Japan develop KD before they reach 10

Incidence of KD in different countries

Singh S et alArch Dis Child 2015

Clinical correlates of KD epidemiology

Country Epidemiological pattern

Japan, Korea, Taiwan (and ?China)

Incidence is continuing to rise

North America, Europe

Incidence has plateaued

Present incidence in Japan is twice the incidence 20 years ago

Arch. Dis. Child 2011

Mean incidence 5.35 / 100,000 children below 5

Singh S, Bhattad S. Kawasaki disease incidence at Chandigarh, North India, during 2009-2014. Rheumatol Int. 2016

Implications for India

May soon replace rheumatic fever to emerge as the commonest cause of acquired heart disease in India

What is new in our basic understanding of the disease?

Kawasaki disease - diagnostic criteria

A. Fever for at least 5 days

B. Presence of 4 of the following 5 conditions:Conjunctival injection Polymorphous rashCervical adenopathy Mucosal changes Acral skin changes:

Acute stage - edema / erythema of hands/feet

Convalescent stage - periungual desquamation

C. Illness not explained by other known

diseases

Kawasaki disease criteria – fallacies

Sensitivity and specificity: not known Incomplete KD

Atypical KD

Kawasaki disease criteria – fallacies

The expanded spectrum of KD – several other systems can be involved

Nagib Dahdah - Montreal, Canada

KD in the very young infants -criteria often not applicable

Kawasaki disease – a perspective

There is currently no diagnostic test for KD

Diagnosis is based on clinical features that are shared with other infectious and inflammatory diseases

Diagnosis and treatment of KD is often delayed or missed, resulting in increased risk of coronary artery aneurysms

Yoshihiro Onouchi

Michael Levin

Jane Burns

Kawasaki disease – the way forward

Kawasaki disease – the way forward

Whole blood gene expression is being evaluated using microarray technology

Gene transcript signatures can possibly distinguish between KD and other febrile illnesses

What is new in imaging of Kawasaki disease?

Role of echocardiography in KD

Fallacies of echocardiography Operator dependent

Inherent fallacies: Only proximal segments of coronary arteries get visualized Lt. Circumflex artery poorly visualized Problems in older children

Dual source CT coronary angiography in Kawasaki Disease 　

Etsuko Tsuda - Osaka, Japan

Role of 128 Slice Dual Source CT Coronary Angiography

Acute KD in a boy aged 11 years

Aneurysms in LAD, LCX and RCA Scanning done at a heart rate of 78/ min.

Acute KD in a boy aged 3 years

Saccular aneurysms in proximal LAD and distal RCA Scanning done at a heart rate of 104 / min.

What is new in treatment of Kawasaki disease?

Treatment of Kawasaki Disease

Disease severity should be assessed

Escalation of therapy:Methylprednisolone (RAISE study 2012)CyclosporineAnakinra

Infliximab

Infliximab for intensification of primary therapy for Kawasaki

disease: a Phase III randomized, double-blind, placebo-controlled

trial

Tremoulet AH, Jain S, Jaggi P, Jimenez-Fernandez S, Pancheri JM, Sun X, Kanegaye JT, Kovalchin JP, Printz BF, Ramilo O, Burns JC.

Lancet 2014

Kawasaki Disease is not merely a one time disease

of childhood

KD has significant long term implications

Cardiovascular imaging in children and adults following Kawasaki disease.

Dietz SM et al. Insights Imaging 2015

Acute myocardial ischemia in adults secondary to missed Kawasaki disease in childhood.

Rizk SR et al. Am J Cardiol 2015

Prevalence of Kawasaki disease in young adults with suspected myocardial ischemia.

Daniels LB et al. Circulation 2012

When children with Kawasaki disease grow up: myocardial and vascular complications in adulthood.

Gordon JB et al. J Am Coll Cardiol 2009

Projections for 2030

Adults with history of having had Kawasaki disease in childhood:

Kawasaki disease will likely impact adult cardiac care programmes

Taiwan 1: 700 populationUnited States 1: 1600 population

Kawasaki disease is no longer a rare cause of acute coronary syndrome presenting in adults

Representative coronary angiograms in four adults at PGI, Chandigarh

KD in the context of a developing country

What else is new in Kawasaki disease?

XI International Kawasaki Disease Symposium, Honolulu, Hawaii: 7 February, 2015

The legend with his family

October 2016

October 2016

‘......Dr. K. is increasingly frail but still has that twinkle in his eye’