Kawasaki disease is an autoimmune vasculitis that primarily affects children under 5 years old. It is characterized by a high persistent fever and changes in the mouth, hands, feet, skin and eyes including redness of the eyes, red cracked lips, strawberry tongue, and a body rash. If left untreated, it can lead to coronary artery aneurysms in 20-25% of cases which increases the risk of heart attack and death. Treatment involves intravenous immunoglobulin and aspirin to reduce fever and risk of aneurysms developing. Long term risks include coronary artery stenosis and aortic root dilation.

1. KAWASAKI DISEASE

2. The disease was first reported by Tomisaku Kawasaki in a four-year-old child with a rash and fever at the
Red Cross Hospital in Tokyo in January 1961.

3. Kawasaki disease affects boys more than girls, with people of Asian ethnicity,
particularly Japanese and Korean people, most susceptible, as well as people of
Afro-Caribbean ethnicity.
Kawasaki disease is also an autoimmune form of vasculitis, and is not associated
with ANCA antibodies, unlike other vasculitis disorders associated with them
(such as granulomatosis with polyangiitis, microscopic polyangiitis, and Churg-
Strauss syndrome.
It is considered to be a necrotizing vasculitis (also called necrotizing angiitis),
which may be identified histologically by the occurrence of necrosis
(tissue death), fibrosis, and proliferation of cells associated with inflammation in
the inner layer of the vascular wall

4. EPIDEMIOLOGY
Recent epidemiologic data show that the incidence of KD in Japan is now 265/100,000 under-five children –
the highest figure ever reported for KD. It is estimated that approximately 1% of children born in Japan
would develop KD by the time they reach 10 years of age.
Korea reports an incidence figure of 134.4 (second highest in the world) while Taiwan reports 82.8 (third
highest in the world) per 100,000 under-five children .
Not only do these three countries have the highest incidence rates in the world, the incidence is also
continuing to increase.
For instance in Japan, the incidence of KD has doubled over the last 20 years.
Incidence rates in Europe and North America are much lower, and vary between 4 to 30/100,000 under-five
children. Further, the incidence rates in these countries have plateaued, and have not shown a discernible
increase over the last two decades.
KD has replaced rheumatic fever to become the commonest cause of acquired heart disease amongst children
in Japan, Northern America and Europe

5. Incidence below 5 years of age
Countary / 100,000
Japan 265
Korea 134
Taiwan 83
Hong kong 74
China Beijing and Sichuan 55 And 7
North America 25
India 4.54 chandi

6. CASE DEFINITION
For epidemiologic surveillance, CDC defines a case of KD as illness in a patient with fever of 5 or more
days duration (or fever until the date of administration of intravenous immunoglobulin if it is given before
the fifth day of fever), and the presence of at least 4 of the following 5 clinical signs:
•Rash
•Cervical lymphadenopathy (at least 1.5 cm in diameter)
•Bilateral conjunctival injection
•Oral mucosal changes
•Peripheral extremity changes
Patients whose illness does not meet the above KD case definition but who have fever and coronary artery
abnormalities are classified as having atypical or incomplete KD.
1. Coronary artery abnormalities include lack of tapering, perivascular brightness, and ectasia Z > 2.5.
2. Echocardiography also may reveal decreased ventricular function, mild valvular regurgitation, and
pericardial effusion.

7. ECHO
•Criteria for Echocardiographic Abnormality in Kawasaki Disease
• LAD or RCA Z score ≥ to 2.5
Patients with large or giant aneurysms (i.e., Z score ≥ 10) are at
the highest risk of both thrombosis and stenosis.
• Coronary artery diameter >3 mm in children <5 years or >4 mm in children ≥
to 5 years
• Lumen diameter ≥1.5 times adjacent segment
• Coronary lumen is clearly irregular
• decreased ventricular function, mild valvular regurgitation, and pericardial
effusion

8. Etio-pathogenesis
Etiology of KD remains an enigma.
Dr Burns describe the occurrence of KD linked to tropospheric wind pattern.
A fungal spore ( high concentration of candida) gets transported across the pacific region by
strong wind blowing through the Upper troposphere. Toxin trigger an abnormal host
immune response in genetically susceptible individuals.
1.The first calcineurin – nuclear factor involved in activation of the T lymphocytes. In this pathway
Single nucleotide polymorphism (SNP) in the gene encoding 1,4,5, inositol trisphosphate 3-kinase C
( ITPKC) results in formation of coronary aneurysm. That why calcineurin inhibitor – cyclosporine is now
proposed for patient refractory to IV IgG.
2. Transforming growth factor- beta signaling pathway. It is belived that SNP in TGFB2, TGFBR2, SMAD3
contributes to coronary aneurysm. That why atorvastatin may benificail in acute stage of KD.
3. Non –synonymous SNP in FCGR@A gene which code for Fcy RIIa has been linked to KD susceptibility.
Variation in gene is responsible for response.

9. American Heart Association (AHA) criteria 2004
A. Fever persisting for at least 5d with
B. At least four of five principal features –
1. Changes in extremities-
Acute – erythema palm, soles, edema hands, feet.
Subacute – periangal peeling of fingers, toes in weeks 2-3
2. Polyporphous exanthema – diffuse maculo-popular, urticarial, erythroderma-multiforme like.
Not vesicular or bullous.
3. Bilateral boulbar conjunctival injection without exudates
4. Changes in lips and oral cavity , erythema, lips cracking, strawberry tonge,diffuse injection of
oral cavity and pharngyeal mucosa.
5. Cervical lymphadenopathy(>1-5cm diameter) usually unilateral
C. Exclusion of other diseases with similar finding e.g. – scarlet fever, viral infection – measeles,
Adenovirus: Stevens- Johnson syndrome, Toxic shock syndrome

10. Clinical criteria of Japanese Kawasaki Disease research comm.
Fever persisting > 5 days
Bilateral conjunctival congestion
Changes of lips and oral cavity
Polymorphous exanthema
Changes of peripheral extremities
Acute nonpurulant cervical lymphadenopathy
Clinical Features :
Acute phase : 10-14 days
Subacute phase : 2-4 weeks - Coronary artery aneurysm (CAA) , arthritis, desquamation
Convalescent phase :6-8 weeks – high ESR, CRP, Thrombocytosis, Beau’s line in nail.

11. Kawasaki disease - AHA diagnostic criteria
Fever of  5 days duration + four of five criteria
Oropharyngeal changes
(90%+ of cases)
1.
Changes in peripheral
extremities
(90%+ of cases)
2.
Cervical
lymphadenopathy
(~75% of cases)
5.
Polymorphous rash
(95%+ of cases)
4.
Bilateral non-purulent
conjunctival injection
(90%+ of cases)
3.

12. Kawasaki disease often begins with a high and persistent fever that is not very
responsive to normal treatment with paracetamol (acetaminophen)
or ibuprofen. It is the most prominent symptom in Kawasaki disease, is a
characteristic sign of the acute phase of the disease, is normally high (above 39-
40 °C), remittent, and is followed by extreme irritability.
Recently, it is reported to be present in patients with atypical or incomplete
Kawasaki disease; nevertheless, it is not present in 100% of cases. The first day
of fever is considered the first day of illness, and the duration of fever is on
average one to two weeks; in the absence of treatment, it may extend for three
to four weeks. Prolonged fever is associated with higher incidence of cardiac
involvement. It responds partially to antipyretic drugs and does not cease with
the introduction of antibiotics. However, when appropriate therapy is started –
intravenous immunoglobulin and aspirin – the fever is gone after two days.

13. KAWASAKI DISEASE SYMPTOMS: RED EYES

14. Bilateral conjunctival inflammation was reported to be the most common
symptom after fever. It typically involves the bulbar conjunctivae, is not
accompanied by suppuration, and is not painful. It usually begins shortly after the
onset of fever during the acute stage of the disease. Anterior uveitis may be
present on slit-lamp examination. Iritis can occur, too. Keratic precipitates are
another eye manifestation (detectable by a slit lamp but are usually too small to
be seen by the unaided eye).
Kawasaki disease presents with set of oral manifestations, the most
characteristic changes are the bright red (erythema), swollen lips (edema) with
vertical cracking (fissures) and bleeding. The mucosa of the oropharynx may be
bright red, and the tongue may have a typical "strawberry tongue" appearance
(marked erythema with prominent gustative papillae). These oral manifestations
are caused by the typical necrotizing microvasculitis with fibrinoid necrosis

15. KAWASAKI DISEASE SYMPTOMS: CHANGES IN THE
LIPS, TONGUE AND MOUTH

16. Cervical lymphadenopathy is seen in 50% to 75% of patients, whereas the other
features are estimated to occur in 90% of patients, but sometimes it can be the
dominant presenting symptom. According to the definition of the diagnostic
criteria, at least one impaired lymph node ≥ 1.5 cm in diameter should be
involved. Affected lymph nodes are painless or minimally painful, nonfluctuant, and
nonsuppurative; erythema of the neighboring skin may occur. Children with fever
and neck adenitis who do not respond to antibiotics should have Kawasaki disease
considered as part of the differential diagnoses

17. KAWASAKI DISEASE SYMPTOMS: BODY RASH

18. In the acute phase of the disease, changes in the peripheral extremities can include erythema of
the palms and soles, which is often striking with sharp demarcation and often accompanied by
painful, brawny edema of the dorsa of the hands or feet. Later, during the convalescent or the
subacute phase, desquamation of the fingers and toes usually begins in the periungual
region within two to three weeks after the onset of fever and may extend to include the palms
and soles. Around 11% of children affected by the disease may continue skin-peeling for many
years.
One to two months after the onset of fever, deep transverse grooves across the nails may
develop (Beau’s lines), and occasionally nails are shed.
The most common cutaneous manifestation is a diffuse macular-papular erythematous rash, which is quite
nonspecific. The rash varies over time and is characteristically located on the trunk; it may further spread
to involve the face, extremities, and perineum. Many other forms of cutaneous lesions have been reported;
they may include scarlatini form, papular, urticariform, multiform-like erythema, and purpuric lesions;
even micro-pustules were reported. It can be polymorphic, not itchy, and normally observed up to the fifth
day of fever.
However, it is never bullous or vesicular

19. KAWASAKI DISEASE SYMPTOMS:
CHANGES TO THE HANDS AND FEET

20. • About a week after the fever breaks, the skin from the fingers and toes may peel off,
sometimes in large pieces. When this happens, new, normal skin shows below the
peeling skin.

21. In the acute stage of Kawasaki disease, systemic inflammatory changes are
evident in many organs.
Joint pain (arthralgia) and swelling, frequently symmetrical, and arthritis can
also occur.
Myocarditis, pericarditis, valvulitis
Diarrhea
Aseptic meningitis
Pneumonitis
Lymphadenitis
and hepatitis may be present and are manifested by the presence of
inflammatory cells in the affected tissues.
If left untreated, some symptoms will eventually relent but coronary artery
aneurysms will not improve, resulting in a significant risk of death or disability
due to myocardial infarction. If treated quickly, this risk can be mostly avoided
and the course of illness cut short.

22. The cardiac complications are the most important aspect of Kawasaki disease. It is the main
cause of heart disease acquired in childhood in the United States and Japan and it appears to
have replaced acute rheumatic fever as the most common cause of acquired heart disease in
children.
Coronary artery aneurysms occur as a sequela of the vasculitis in 20-25% of untreated children.
It is first detected at a mean of 10 days of illness and the peak frequency of coronary artery
dilation or aneurysms occurs within four weeks of onset.
Aneurysms are classified into
small (internal diameter of vessel wall <5 mm),
medium (diameter ranging from 5–8 mm), and
giant (diameter > 8 mm).
Saccular and fusiform aneurysms usually develop
between 18 and 25 days after the onset of illness.

23. Even when treated with high-dose IVIG regimens within the first 10 days of illness, 5% of
children with Kawasaki disease develop at the least transient coronary artery dilation and 1%
develop giant aneurysms.
Death can occur either due to myocardial infarction secondary to blood clot formation in a
coronary artery aneurysm or to rupture of a large coronary artery aneurysm. Death is most
common between two to 12 weeks after the onset of illness.

24. Many risk factors predicting coronary artery aneurysms have been identified,
including persistent fever after IVIG therapy,
low hemoglobin concentrations,
low albumin concentrations,
high white-blood-cell count,
high band count,
high CRP concentrations,
male sex, and
age less than one year.
Coronary artery lesions resulting from Kawasaki disease change dynamically with time. Resolution
one to two years after the onset of the disease has been observed in half of vessels with
coronary aneurysms. Narrowing of the coronary artery, which occurs as a result of the healing
process of the vessel wall, often leads to significant obstruction of the blood vessel and lead
to the heart not receiving enough blood and oxygen. This can eventually lead to heart muscle
tissue death (myocardial infarction).
MI caused by thrombotic occlusion in an aneurysmal, stenotic, or both aneurysmal and stenotic
coronary artery is the main cause of death from Kawasaki disease. The highest risk of MI occurs
in the first year after the onset of the disease. MI in children presents with different symptoms
from those in adults. The main symptoms were shock, unrest, vomiting, and abdominal pain; chest
pain was most common in older children

25. The course of the disease can be divided into three clinical phases.
•The acute febrile phase, which usually lasts for one to two weeks, is characterized by
fever, conjunctival injection, erythema of the oral mucosa, erythema and swelling of the
hands and feet, rash, cervical adenopathy, aseptic meningitis, diarrhea, and hepatic
dysfunction. Myocarditis is common during this time, and a pericardial effusion may be
present. Coronary arteritis may be present, but aneurysms are generally not yet visible
by echocardiography.
•The subacute phase begins when fever, rash, and lymphadenopathy resolve at about one to
two weeks after the onset of fever, but irritability, anorexia, and conjunctival injection
persist. Desquamation of the fingers and toes and thrombocytosis are seen during this
stage, which generally lasts until about four weeks after the onset of fever. Coronary
artery aneurysms usually develop during this time, and the risk for sudden death is highest.
•The convalescent stage begins when all clinical signs of illness have disappeared, and
continues until the sedimentation rate returns to normal, usually at six to eight weeks after
the onset of illness.

29. Valvular insufficiencies, particularly of mitral or tricuspid valves, are often
observed in the acute phase of Kawasaki disease due to inflammation of the
heart valve or inflammation of the heart muscle-induced myocardial dysfunction,
regardless of coronary involvement. Aortic root dilation is a known finding in the
acute/subacute phase of Kawasaki disease (KD). These changes may be
inflammatory and related to the degree of coronary artery (CA) dilation, and may
lag when compared to CA changes. Long-term changes in the aorta have not been
previously reported.
Long-term Aortic Root Dilation in Kawasaki Disease with Coronary Aneurysm
1.Michael Carr;
2.Caroline R Schuette;
3.Elfriede Pahl
Am J Cardiol. 2001 Apr 1;87(7):919-22.
Aortic root dilation in Kawasaki disease.
Ravekes WJ1, Colan SD, Gauvreau K, Baker AL, Sundel RP, van der Velde ME, Fulton DR, Newburger JW.

30. The disease often compromises vessels from the intima layer to the perivascular area, forming
aneurysms in several different stages in a childhood poly arteritis.
The most severe complication of the disease is coronary vaculitis, leading to coronary artery
changes and affecting 15%-20% of patients if left untreated. These changes include aneurysms,
coronary artery ectasias and stenosis, responsible for 2% of mortality. KD results in focal
abnormalities only at the site of aneurysms. Vessels proximal and distal to the lesion appear healthy
and have a smooth luminal surface and normal diameter.
It may be associated with aneurysms of other arteries that is aorta, the abdominal aorta; axillary
artery; brachiocephalic artery; iliac and femoral arteries and renal artery.
Aortic aneurysms should also be assessed and the aortic diameter should be measured with imaging
studies because there are evidence the aorta dilation is common in patients with KD. This dilations
do not regress over the first year of the disease.
Ischemic necrosis of the distal extremities is a rare but potentially severe complication of KD.

31. Investigations:
Blood tests: -
•Complete blood count may reveal normocytic anemia and eventually thrombocytosis.
•Erythrocyte sedimentation rate will be elevated.
•C-reactive protein will be elevated.
•Liver function tests may show evidence of hepatic inflammation and low serum albumin levels.
Other optional tests include:
•Electrocardiogram may show evidence of ventricular dysfunction or, occasionally, arrhythmia due to
myocarditis.

32. CARDIOVASCULAR MANIFESTATIONS OF
ACUTE KAWASAKI DISEASE
• EKG changes
• Arrhythmias
• Abnormal Q waves
• Prolonged PR and/or QT intervals
• Low voltage
• ST-T–wave changes.
• CXR–cardiomegaly

33. Investigations:
Blood tests: -
•Echocardiogram may show subtle coronary artery changes or, later, true aneurysms.
•Ultrasound or computerized tomography may show hydrops (enlargement) of the
•gallbladder.
•Urinalysis may show white blood cells and protein in the urine (pyuria and proteinuria) without evidence of
bacterial growth.
•Lumbar puncture may show evidence of aseptic meningitis.
•Angiography was historically used to detect coronary artery aneurysms, and remains the gold standard for
their detection, but is rarely used today unless coronary artery aneurysms have already been detected by
echocardiography.
•Temporal artery biopsy

34. Echo finding : perivascular brightnes
Absence of tapperingin coronaries
Decrease LV function
Pericardial effusion
Mitral regurgitation.
Dilated aortic root.
And Z score for left anterior or right coronary between 2-2.5
With early treatment, rapid recovery from the acute symptoms can be expected, and the risk of coronary
artery aneurysms is greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited
(i.e. the patient will recover eventually), but the risk of coronary artery involvement is much greater.
Overall, about 2% of patients die from complications of coronary vasculitis
Z score Interpretation
<2 Normal
2-2.5 Mild dilatation or ectasia
2.5-5 Small coronary aneurysm
5-10 Medium
> 10 Giant aneurysm
RV
RA
LV

35. TRICUSPID REGURGITATION

37. HARADA RISK SCORE :
WBC > 12000/Cmm
Platelets count : 12000/Cmm
CRP + >3
Hematocrit : 35%
Albumin : 3.5gm /dl
Age 12 months
Sex Male
Give IVIG to children who fulfil 4 of the Above criteria, assess with in
9 days Of onset of illness

38. TREATMENT
• IVIG 2g/kg over 12 hours
• Aspirin 30-50 mg/kg/day (4 divided doses)
– Then 2-5 mg/kg/od 48 hours after fever settles
• Fever recrudescence/IVIG resistance:
– Repeat IVIG
– OR corticosteroids
– OR Infliximab
Egami defined “responder” as a patient in whom fever disappeared and CRP dropped by more
than 50% within 48 h after IVIG

39. Preliminary case definition of Covid -19 Kawasaki disease
Children and adolescents 0–19 years of age with fever > 3 days
AND two of the following:
1.Rash or bilateral non-purulent conjunctivitis or muco-cutaneous inflammation signs (oral, hands or feet).
2.Hypotension or shock.
3.Features of myocardial dysfunction, pericarditis, valvulitis, or coronary abnormalities ( including ECHO
findings or elevated Troponin/NT- proBNP),
4.Evidence of coagulopathy (by PT, PTT, elevated d-Dimers).
5.Acute gastrointestinal problems (diarrhoea, vomiting, or abdominal pain).
AND
Elevated markers of inflammation such as ESR, C-reactive protein, or procalcitonin.
AND
No other obvious microbial cause of inflammation, including bacterial sepsis, staphylococcal or streptococcal
shock syndromes.
AND
Evidence of COVID-19 (RT-PCR, antigen test or serology positive), or likely contact with patients with
COVID-19.

40. RISK FACTORS FOR REFRACTORY KD
Risk factors for refractory KD, includes-
High neutrophil count; low hemoglobin; thrombocytopenia; low albumin; high CRP; high lactate
dehydrogenase; hyponatremia; elevated AST; abnormal findings in initial echocardiography; and
recurrent KD

41. Egami Risk Score
A cut-off point ≥3 identifies patients with Kawasaki syndrome at high risk of being resistant to intravenous immunoglobulin
Points
Age <6 months 1 point
Days of illness <4 1 point
Platelet count <300 × 109
/L 1 point
C-reactive protein >8 mg/dL 1 point
Alanine aminotransferase >80 IU/L 2 points
Kobayashi Risk Score
A cut-off point ≥4 identifies patients with Kawasaki syndrome at high risk of being resistant to intravenous immunoglobulin
Points
Sodium ≤133 mmol/L 2 points
Days of illness at initial treatment ≤4 2 points
Aspartate aminotransferase ≥100 IU/L 2 points
Percentage of neutrophils ≥80% 2 points
C-reactive protein ≥10 mg/dL 1 point
Age ≤12 months 1 point
The latest scoring systems evaluating non-responsiveness to treatment with intravenous
immunoglobulin in children with Kawasaki syndrome: the Egami and Kobayashi scores were primed
in 2006, the Sano score in 2007.

42. Items Egami Score Kobayashi Score Sano Score
C-reactive protein ■ ■ ■
Age ■ ■
Days of illness ■ ■
Alanine
aminotransferase
■
Total bilirubin ■
Aspartate
aminotransferase
■ ■
Sodium ■
Percentage of
neutrophils
■
Platelet count ■ ■
Items evaluated in the different risk scores for Kawasaki syndrome.

43. Clinical Studies Egami Score Kobayashi Score Sano Score
Year of publication 2006 2006 2007
Population Japanese Japanese Japanese
Sample size 320 patients 750 patients 112 patients
Diagnosis of Kawasaki syndrome Japanese criteria
Revision of the Japanese criteria
(5th edition)
Criteria not specified
Diagnosis of coronary artery
abnormalities
Not adjusted for body surface
area
Not adjusted for body surface
area
Adjusted for body surface area
(according to de Zorzi’s criteria)
Treatment with intravenous
immunoglobulin (IVIG)
Single 2 g/kg/dose within 9 days
of illness
1 g/kg per day for 2 consecutive
days
1 g/kg per day (within 2 days of
illness) for 2 consecutive days
Definition of non-responsiveness
to intravenous immunoglobulin
(IVIG)
Persistent fever (≥37.5 °C) and
fall in C-reactive protein by less
than 50% within 48 h after IVIG
treatment
Persistent fever (≥37.5°C)
lasting more than 24 h or
recrudescent fever (after an
afebrile period) associated with
disease symptoms
Persistent fever (≥37.5 °C) over
24 h after finishing IVIG
infusion
Sensitivity
78% **
61% *
86% ** 77% **
Differences among the clinical studies for which the latest risk scores for Kawasaki syndrome were formulated.

44. "INCOMPLETE KAWASAKI DISEASE”
Recently, the term "atypical Kawasaki disease" has been used to describe patients with incomplete
presentation of the disease, regardless of the presence of coronary complications and is exchangeable
for "incomplete Kawasaki disease”.

45. RATIONALE FOR BLOCKING TNF-Α IN KD
• Murine model of KD:
• Production of TNF- α in the peripheral immune system
• Response site directed, migration to the coronary arteries
• Mice treated with etanercept and TNFRI knockout mice
resistant to development of both coronary arteritis and
coronary aneurysm formation.
Hui-Yuen JS et al J Immunol. 2006;176:6294-301

46. INFLIXIMAB
• Chimeric monoclonal antibody against TNFα
• Dose 6mg/kg IV
• Repeat after 2 weeks if ongoing inflammation (consider differential diagnosis)

47. KD OUTCOME
• 25 % CAA untreated
• 4-9% CAA with IVIG and aspirin
• IVIG resistance 15-20%
– Very high risk of CAA: 20-30%
• The acute mortality rate due to myocardial infarction <1%

48. PATIENT FOLLOW-UP CATEGORIES
• Five categories based on coronary arteries findings
• No coronary changes at any stage of illness
• Transient CA ectasia, resolved within 6-8 wks
• Small/medium solitary coronary aneurysm
• One or more large or giant aneurysms or multiple smaller/complex aneurysms in same CA,
without obstruction
• Coronary artery obstruction

49. MANAGEMENT CATEGORIES
• Pharmacologic therapy
• Physical activity
• Follow-up and diagnostic testing
• Invasive testing

50. I. NO CORONARY CHANGES AT ANY STAGE OF ILLNESS
• Pharmacologic Therapy
• None beyond 6-8 weeks
• Physical Activity
• No restrictions beyond 6-8 weeks
• Follow-up and diagnostic testing
• CV risk assessment, counseling @ 5 yr intervals
• Invasive testing
• None recommended

51. II. TRANSIENT CA ECTASIA, RESOLVED WITHIN 6-8 WKS
• Pharmacologic Therapy
• None beyond 6-8 weeks
• Physical Activity
• No restrictions beyond 6-8 weeks
• Follow-up and diagnostic testing
• CV risk assessment, counseling @ 5 yr intervals
• Invasive testing
• None recommended

52. III. SINGLE SMALL OR MEDIUM SIZE ANEURYSM
• Pharmacologic Therapy
• Low dose ASA until regression documented
• Physical Activity
• None beyond 1st 6-8 weeks in patients <11 y.o.
• 11-20 y.o.: Restrictions based on biennial stress test/myocardial perfusion scan
• Contact/high-impact discouraged if taking anti-plt drugs
• Follow-up and diagnostic testing
• Annual exam, echo, EKG
• CV risk assessment, counseling
• Invasive testing
• Angiography if suggestion of ischemia

53. IV. ANEURYSMS WITHOUT STENOSIS
• Pharmacologic Therapy
• Long-term antiplatelet tx & warfarin or LMWH
• Physical Activity
• Restrictions based on stress test/myocardial perfusion scan
• Contact/high-impact avoided due to risk of bleeding
• Follow-up and diagnostic testing
• Biannual exam, echo, EKG
• Annual stress test/myocardial perfusion scan
• Invasive testing
• Angiography @ 6-12 mos, sooner/repeated if clinically indicated
• Elective repeat in certain circumstances

54. V. OBSTRUCTION
• Pharmacologic Therapy
• Long-term low-dose ASA, ± warfarin or LMWH if giant aneurysm persists
• Consider ß-blockade to reduce myocardial O2 consumption
• Physical Activity
• No contact or high impact sports
• Other activity guided by stress testing or perfusion scan
• Follow-up and diagnostic testing
• Biannual exam, echo and EKG
• Annual stress test/myocardial perfusion scan
• Invasive testing
• Angiography indicated to assess lesions and guide therapy. Repeat angiography
with change in symptoms.

55. CARRY HOME MESSAGES
• HSP and KD are the commonest paediatric vasculitides
• KD still kills children- limited window of opportunity to
influence outcome: switch off the inflammation ASAP
– Earlier use of steroids and infliximab ie after 1 dose of IVIG
• Polygenic and no infectious cause yet found
• Does KD predispose to premature atherosclerosis?