what is new - kawasaki disease : recent trends - prof surjeet singh
TRANSCRIPT
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’