Kawasaki disease. Dr Laurence Lacroix

Transcription

1 Dr Laurence Lacroix

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3 DEFINITION: o Kawasaki disease(kd) is an acute febrile illness of childhood ovasculitisof medium-sized extraparenchymal arteries o Predilection for coronary arteries o Leading cause of acquired heart disease in developed countries 3

4 HISTORY: o1st describedin 1967 by a Japanese pediatrician (Dr TomisakuKawasaki) (Kawasaki T «Acute febrile mucocutaneoussyndrome with lymphoid involvement with specific desquamation of the fingers and toes in children» Jpn J Allergy (Arerugi) 1967;16: ) o Mucocutaneous lymph node syndrome o Cardiac involvement not apparent at first oautopsy cases: coronary artery aneurysms and thrombosis okd described in children around the world and virtually all races (Japan +++) 4

5 EPIDEMIOLOGY: Kawasaki disease o Nationwide surveys every 2 years in Japan oworldwidelinearincreasein incidence rate since1990 s (diseaseawarenessand recognition?) o2010: Incidence rate in Japan: 240/ (0-4 y old), in the USA 20/ oheightened incidence in people of Asian descent, both inside and outside Asia. 5

6 EPIDEMIOLOGY: Kawasaki disease opeakincidence between6 and 11 months o80-90% < 5 years oboys > Girls 6

7 ETIOLOGY: Unknown! Kawasaki disease Genetic susceptibility -Asian and Asian- American populations -family members of an index case Impaired immunoregulation Infectious agents, toxins KD 7

8 ETIOLOGY: J Infect Jul;67(1):1-10 8

9 CLINICAL DIAGNOSIS: based on clinical criteria! ofever o Usually more than 39.9 C, for at least 5 days o High spiking and remittent o Persists 1-2 weeks without treatment o May not respond to antipyretics o Usually resolves in 1-2 days after treatment with immunoglobulins o Altered general appearance o Featuresof KD canfluctuate: thorough medical history to determine their presence duringthe periodof illness. 9

10 CLASSIC CLINICAL CRITERIA (according to the American Heart Association) ofever of atleast 5 days duration opresenceof atleast 4of the following principal features: 1. Bilateral conjunctival injection 2. Changes in lipsand oral cavity 3. Cervical lymphadenopathy 4. Polymorphous exanthem 5. Changes in the extremities o Exclusion of other diseases with similar findings 10

11 1. Bilateral nonexudative bulbar conjunctivitis 11

12 2. Oropharyngeal inflammation Cracked lips Strawberry tongue + diffuse erythema of the oropharyngeal mucosa

13 3. Cervical lymph nodes enlargement Unilateral(> 1.5 cm)

14 4. Maculopapular rash Kawasaki disease

15 5. Changes in the extremities Erythemaof palms and/or soles, edema of hands and/or feet. Hand and/or foot periungual peeling skin(week 2-3 of disease course).

16 ASSOCIATED CLINICAL FEATURES: Adapted from Circulation. 2004;110(17):

17 Erythema and induration at BCG site

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20 COMPLICATIONS: Kawasaki disease oprognosis based on the presence and severity of coronary artery lesions o Mild dilation giant aneurysms o 3-5% of children receiving IVIG oup to 25%in untreated children omore frequent in children < 6 months and 5 years 20

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22 DIFFERENTIAL DIAGNOSIS: oviral infection measles adenovirus enterovirus Epstein-Barr virus obacterialinfection Scarlet fever cervical lymphadenitis o Drug hypersensitivity o Stevens-Johnson syndrome 22

23 DIFFERENTIAL DIAGNOSIS: o Juvenile idiopathic arthritis(systemic onset) otoxicshocksyndrome (associated with Staphylococcus or Streptococcus) o Staphylococcal scalded skin syndrome (SSSS) o Others: acrodynia(mercury toxicity) 23

24 LABORATORY FINDINGS: o sedimentationrate (> 40 mm/h) o CRP o neutrophilia with immature forms othrombocytosis(> 1 week, peaksat3 weeks) oanemia o hypoalbuminemia o liverenzymes o sterile pyuria 24

25 ECHOCARDIOGRAPHY: Kawasaki disease o coronary artery dimensions o myocardial function o valve regurgitation o pericardial effusion echo echo echo echo diagnosis 1-2 weeks 6 weeks 12 months Time from diagnosis 25

26 POOR CLINICAL OUTCOME RISK FACTORS: o< 6 months, > 9 years o Asian, Pacific Islander and Hispanic races olaboratory parameters: o Neutrophilia o Thrombocytopenia o Hyponatremia o Elevated CRP o Elevated liver tests 26

27 INCOMPLETE KAWASAKI DISEASE: o More frequentin children< 12 m and > 5 y old o classic features of KD but not enough to meet the case definition. o < 4of the principal features but suggesting laboratory results or echocardiography o approximately 25% of the KD cohort o risk of coronary artery complications (diagnostic and treatment delay) 27

28 4) Supplemental laboratory criteria: -albumin 3.0 g/dl -anemia for age -elevation of ALT -platelets after 7 days 450,000/mm3 -white blood cell count 15,000/mm3 -urine 10 WBC/highpower field 28

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30 COMPLEMENTARY EXAMINATION: o Blood: o Complete blood count (CBC): o Anemia o Leukocytosis (elevated white blood cells /µL) o Thrombocytosis= elevated platelets ( /µL after 7 days of illness) o Elevated erythrocyte sedimentation rate o Elevated C-reactive protein 30

31 COMPLEMENTARY EXAMINATION: o Blood: o AST, ALT, albumin, sodium: elevated liver function tests, decreased albumin and natremia(capillary leak) o Abnormal lipids decreasedlevelsof total cholesterol, apolipoprotein A1 and high-density lipoprotein o Serology: measles, EBV, ASLO o Blood culture if suspicion of septicemia 31

32 COMPLEMENTARY EXAMINATION: o Urine: Urine test strip: sterile pyuria (presence of leukocytes with negative urine culture) 32

33 COMPLEMENTARY EXAMINATION: o Stools: o o Culture for enterovirus, adenovirus: if positive, Kawasaki disease unlikely Search for blood may be positive 33

34 COMPLEMENTARY EXAMINATION: o Echocardiography: Myocarditis Pericardial effusion Congestive heart failure Valvular regurgitation (mitral> aortic) Coronary arteries abnormalities 34

35 COMPLEMENTARY EXAMINATION: o Other: o Prepare a throat swab for o measles PCR o Group A ß-hemolytic streptococcus culture if positive, Kawasaki disease unlikely 35

36 CASE DEFINITION-Major criteria: o o Fever 5 days Clinical criteria(signs of mucocutaneous inflammation) 1. Non-exudative bilateral conjunctivitis 2. Involvement of the lipsand oral cavity: 3. erythema, cracked lips, strawberry red tongue, diffuse inflammation of the oral and pharyngeal mucosa. Usually unilateral neck lymphadenopathy( 1.5 cm) 4. Polymorphic exanthem 5. Involvement of handsand feet: erythemaof palms and/or soles, edema of hands and/or feet. Hand and/or foot periungual peeling skin(week 2-3 of disease course) 36

37 CASE DEFINITION-Minorcriteria: o Erythrocyte sedimentation rate 40 mm/h OR CRP 3 mg/dl o Echocardiographyshowing coronary artery or heart involvement 37

38 DIAGNOSTIC CERTAINTY LEVELS: 38

39 DIAGNOSTIC CERTAINTY LEVELS: 39

40 ACUTE PHASE TREATMENT: IVIG Intravenous Immunoglobulins (IVIG) 2g/kg 012/11/23/ oshould be instituted less than 10daysafter fever onset o Aim at reducing the inflammation o2 nd dose 2g/kg if refractory disease (persistent or recurrent fever at least 36 hours after the 1 st IVIG dose has been infused-11% to 23% of patients) 40

41 ACUTE PHASE TREATMENT: Aspirin Aspirin (acetylsalicylic acid) mg/kg/day o Anti-inflammatory dosage odivided into 4doses/day ountil the child is afebrile 41

42 ACUTE PHASE TREATMENT: oin case of resistance to IVIG after a 2 nd dose, consider a 3-day course of 30 mg/kg/day intravenous pulse of methylprednisolone 42

43 CONVALESCENT PHASE TREATMENT: oaspirin (acetylsalicylic acid)3 5 mg/kg per day in once-daily dosing(antithrombotic dosage) omaintain it until the patient shows no evidence of coronary changes by 6 to 8 weeks after the onset of illness ofor children who develop coronary abnormalities, aspirin may be continued indefinitely 43

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45 SUGGESTED READING: Kawasaki disease. Son MB, Newburger JW. Pediatr Rev Apr;34(4): Kawasaki disease Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionalsfromthe Committeeon RheumaticFever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Newburgeret al. Committeeon RheumaticFever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics Dec;114(6): Kawasaki disease: part I. Diagnosis, clinical features, and pathogenesis. Bayers S, Shulman ST, Paller AS. J Am AcadDermatol Oct;69(4):501.e1-11; quiz doi: /j.jaad Review. Kawasaki disease: part II. Complications and treatment. Bayers S, Shulman ST, Paller AS. J Am AcadDermatol Oct;69(4):513.e1-8; quiz doi: /j.jaad Review. 45

46 QUESTIONS 46