Kawasaki Disease            Androu Waheeb      RCSI-Bahrain Medical Student            Acknowledgement:Dr. Brian McCrindle...
History Tomisaku   Kawasaki, Red Cross Hospital,  Tokyo, Japan (Jan 1961) Alternative Names   lymphnode syndrome   Muc...
Definition Auto-immune    mediated vasculitis   Medium  sized arteries   Favours coronary arteries
Epidemiology 85%   younger than 5 years Uncommon in 1 year < age < 5 years Male:female ratio 1.6:1 2-5 Increased preva...
Epidemiology cont’d Average recurrence 3% Average sibling occurrence 1% Average mortality < 1% Racial Demographic   A...
Aetiology Unknown:    infectious pattern Virus Bacterial(superantigenic toxin vs.  conventional antigen) Genetics Aut...
6 cardinal symptoms/signs1.       Fever     •     ≥ 5 days     •     ≥ 40oC     •     self-remitting     •     onset1.    ...
6 cardinal symptoms/signs cont’d3.       Oromucosal changes     •    Lips             Red             Cracked          ...
6 cardinal symptoms/signs cont’d5.       Changes in extremities     •     Erythema of hands and feet             a/w pain...
6 cardinal symptoms/signs cont’d6.       Cervical lymphadenopathy     •    Least common     •    ≥ 1 lymph node, ≥ 1.5 cm ...
Diagnostics No   specific diagnosis or test   Use clinical presentation   Not always simultaneous
Diagnostic Criteria Japanese     MOH   Complete     KD    Fever   + 5/6 cardinal symptoms   Incomplete   KD    Fever ...
Cardiac Findings    Prominent in the acute phase    leading cause of long-term morbidity and     mortality.    Involvem...
Cardiac Findings cont’d Cardiac      auscultation      hyperdynamic precordium      Tachycardia      a gallop rhythm  ...
Non-Cardiac Findings increased    irritability vs. other febrile  diseases Arthritis or arthralgia (first week)   multi...
Non-Cardiac Findings cont’d Hepatic enlargement + jaundice Acute acalculous gallbladder distension   first        two w...
Non-Cardiac Findings cont’d siteof previous BCG erythema +  induration   Common   Younger   than 1 year perianal   ery...
Characteristic Lab Findings   acute stage (4-6w)       Leukocytosis            predominance of immature and mature gran...
Lab Findings cont’d serum transaminases moderately elevated Hypoalbuminemia   a/w   with increased severity + duration...
Treatment Conventional=IVIG     + Aspirin IVIG   Reduces    the prevalence of coronary artery    abnormalities   MOA u...
Treatment cont’d Aspirin   Anti-platelet                and anti-inflammatory; no reduction of    CAA incidence   80-10...
Treatment failure Retreatment  IVIG 2g/kg Methylprednisolone IV pulse 30mg/kg 2-3  h od 1-3 d Infliximab (monoclonal an...
Treatment Failure cont’d Ulinastatin Plasmapheresis Cytotoxic   agents   Cyclophosphamide   Cyclosporin   A MTX:  di...
Management Serialstress test Follow up 4-6 w, 6m, 1 y   Echo 20%   AneurysmstenosisMI   Percutaneous   Transluminal...
Complications   Cardiac       Coronary artery dilations and aneurysms (5%)          Thrombosis          Myointimal pro...
Coronary Aneurysms Smaller with time Risk factors   Male  8 year < Age < 1 year   Fever non-responsive to IVIG   Dec...
Morbidity & Mortality Thrombosis   principal cause MI highest rate 1 year after onset   LMCA   RCA   + LAD Nocardiac ...
Take Home Message Auto-immune     mediated vasculitis  (mucuopurulent lymphadenopathy) 6 Cardinal Symptoms, don’t have t...
References1.   Kim, D. S. (2006). "Kawasaki Disease." Yonsei Medical Journal 47(6): 759-772.2.   Li, X.-h., X.-j. Li, et a...
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Kawasaki Disease

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Overview of Kawasaki Disease

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  • Infectious pattern: peak at 1 y and v. little before and after 5; seasonal variation;
  • E: swelling of the dorsa of the feet + erythema of soles H: perianal desquamation
  • Help identify KD
  • Antinuclear antibody, infections, cancer, lung disease, gastrointestinal diseases, hormonal diseases, blood diseases, skin disease, elderly people, family history of rheumatic disease. 5% occurrence naturally Rheumatoid Factor for RA Pleocytosis: more than regular number of cells in CSF
  • Stenotic lesions
  • Kawasaki Disease

    1. 1. Kawasaki Disease Androu Waheeb RCSI-Bahrain Medical Student Acknowledgement:Dr. Brian McCrindle at the Hospital for Sick Children, Toronto
    2. 2. History Tomisaku Kawasaki, Red Cross Hospital, Tokyo, Japan (Jan 1961) Alternative Names  lymphnode syndrome  Mucocutaneous lymph node syndrome  Kawasaki syndrome Syndrome ?!
    3. 3. Definition Auto-immune mediated vasculitis  Medium sized arteries  Favours coronary arteries
    4. 4. Epidemiology 85% younger than 5 years Uncommon in 1 year < age < 5 years Male:female ratio 1.6:1 2-5 Increased prevalence in Asian decent (10x)  Japan highest (2006: 188.1/100k < 4 yo) 6  Korea 2nd (2005: 104.6/100k < 5 yo)7  Vs USA (2000:17.1/100k < 5 yo) 8
    5. 5. Epidemiology cont’d Average recurrence 3% Average sibling occurrence 1% Average mortality < 1% Racial Demographic  Asian and Pacific Island descent > non-Hispanic African-American > Hispanics > Caucasian
    6. 6. Aetiology Unknown: infectious pattern Virus Bacterial(superantigenic toxin vs. conventional antigen) Genetics Auto-immune
    7. 7. 6 cardinal symptoms/signs1. Fever • ≥ 5 days • ≥ 40oC • self-remitting • onset1. Non-purulent bilateral conjunctivitis • Onset after fever
    8. 8. 6 cardinal symptoms/signs cont’d3. Oromucosal changes • Lips  Red  Cracked  Bleeding  Swollen • Strawberry tongue3. Erythematous rash • Truncal peripheral extremities
    9. 9. 6 cardinal symptoms/signs cont’d5. Changes in extremities • Erythema of hands and feet  a/w pain  a/w brawny oedema dorsum of hand • Desquamation of fingers and toes
    10. 10. 6 cardinal symptoms/signs cont’d6. Cervical lymphadenopathy • Least common • ≥ 1 lymph node, ≥ 1.5 cm in diameter • Unilateral • Anterior cervical triangle
    11. 11. Diagnostics No specific diagnosis or test  Use clinical presentation  Not always simultaneous
    12. 12. Diagnostic Criteria Japanese MOH  Complete KD Fever + 5/6 cardinal symptoms  Incomplete KD Fever + 4/6 cardinal symptoms + evidence of coronary artery abnormality  Echo: ≥ 3mm ≤ 5 yo; ≥ 4mm ≥ 5 yo  Internal diameter >1.5 x from adjacent segment More common in younger children
    13. 13. Cardiac Findings Prominent in the acute phase leading cause of long-term morbidity and mortality. Involvement of 1. Pericardium 2. Myocardium 3. Endocardium 4. Valves 5. Coronary arteries
    14. 14. Cardiac Findings cont’d Cardiac auscultation  hyperdynamic precordium  Tachycardia  a gallop rhythm  innocent flow murmur  Anemia  Fever  depressed myocardial contractility (myocarditis) significant MR  pansystolic regurgitation murmur low cardiac output syndrome or shock (poor myocardial function)
    15. 15. Non-Cardiac Findings increased irritability vs. other febrile diseases Arthritis or arthralgia (first week)  multiplejoints  favours knees and ankles. Gastrointestinal complaints (33%)  Diarrhoea  Vomiting  abdominal pain, occur in approximately
    16. 16. Non-Cardiac Findings cont’d Hepatic enlargement + jaundice Acute acalculous gallbladder distension  first two weeks  Cause hydrops  identified by abdominal ultrasound
    17. 17. Non-Cardiac Findings cont’d siteof previous BCG erythema + induration  Common  Younger than 1 year perianal erythematous desquamation
    18. 18. Characteristic Lab Findings acute stage (4-6w)  Leukocytosis  predominance of immature and mature granulocytes  Upregulated apoptosisDepleted peripheral lymphocytes.  Anemia  RBC indices,  particularly with a prolonged duration of active inflammation.  Elevated Acute Phase Reactants  ESR  CRP  Platelet count rapidly increases week 2-3, (can be > 1,000,000/mm3)  ANA and RF -ve
    19. 19. Lab Findings cont’d serum transaminases moderately elevated Hypoalbuminemia  a/w with increased severity + duration Intermittent sterile pyuria  (Urinalysis not suprapubic)urethritis CSF pleocytosis  mononuclear cells predominance  normal glucose and protein Inflammatory synovial fluid Decreased plasma cholesterol, HDL, apolipoprotein A-I
    20. 20. Treatment Conventional=IVIG + Aspirin IVIG  Reduces the prevalence of coronary artery abnormalities  MOA unknown (neutralization of antigen or immune inhibition?)  2g/kg infusion 10-12 h  Higher dose  Less infusions  Best within 7 days (earlier better)  Live vaccines 11 mo reduced immunogenicity
    21. 21. Treatment cont’d Aspirin  Anti-platelet and anti-inflammatory; no reduction of CAA incidence  80-100 mg/kg/d/4 doses  3-5 mg/kg/d afebrile 72 hours + ≥ day 14 of illness.  D/C no CAA 6 week follow-up  Reye syndrome a/w varicella and influenza vaccine Steroids  Controversial Combined (30mg/kg steroids + conventional)  No significant
    22. 22. Treatment failure Retreatment IVIG 2g/kg Methylprednisolone IV pulse 30mg/kg 2-3 h od 1-3 d Infliximab (monoclonal anti-TNF-a antibody)
    23. 23. Treatment Failure cont’d Ulinastatin Plasmapheresis Cytotoxic agents  Cyclophosphamide  Cyclosporin A MTX: dihydrofolate reductase inhibitor (anti-inflammatory)  10mg/BSA, once weekly  MOA unclear
    24. 24. Management Serialstress test Follow up 4-6 w, 6m, 1 y  Echo 20% AneurysmstenosisMI  Percutaneous Transluminal Angioplasty  Rotational atherectomy  Stent  Bypass graft  transplant
    25. 25. Complications Cardiac  Coronary artery dilations and aneurysms (5%)  Thrombosis  Myointimal proliferation  stenosis (20%)myocardial ischemia  1% giant aneurysms  Aspirin, clopidogrel, ticlopidine  LMW Heparin, Warfarin, anti platelet receptor monoclonal antibodies  Pericarditis + effusion  Myocarditis  Valvulitis (1%. Mitral Valve)
    26. 26. Coronary Aneurysms Smaller with time Risk factors  Male 8 year < Age < 1 year  Fever non-responsive to IVIG  Decreased  Hb  Serum[albumin]  Increased  WBC  Band  CRP
    27. 27. Morbidity & Mortality Thrombosis principal cause MI highest rate 1 year after onset  LMCA  RCA + LAD Nocardiac sequelae within 1st mo  full recovery + asymptomatic
    28. 28. Take Home Message Auto-immune mediated vasculitis (mucuopurulent lymphadenopathy) 6 Cardinal Symptoms, don’t have to occur in totality (Japanese Diagnostic Criteria) Etiology unknown: behaves as infectious and genetic Devastating cardiac complications  MI, anurysms, vasculitis Treatment: IVIG + Aspirin + Complications
    29. 29. References1. Kim, D. S. (2006). "Kawasaki Disease." Yonsei Medical Journal 47(6): 759-772.2. Li, X.-h., X.-j. Li, et al. (2008). "Epidemiological Survey of Kawasaki Disease in Sichuan Province of China." Journal of tropical pediatrics 54(2): 133-136.3. Huang, W.-C., L.-M. Huang, et al. (2009). "Epidemiologic Features of Kawasaki Disease in Taiwan, 2003-2006." Pediatrics 123(3): e401-405.4. Chang, R.-K. R. (2002). "Epidemiologic characteristics of children hospitalized for Kawasaki disease in California." Pediatric Infectious Disease Journal 21(12): 1150-1155.5. Morens, D. M., L. J. Anderson, et al. (1980). "National Surveillance of Kawasaki Disease." Pediatrics 65(1): 21-25.6. Yosikazu Nakamura, M. Y., Ritei Uehara, Izumi Oki, Makoto Watanabe, and Hiroshi Yanagawa (2008). "Epidemiologic Features of Kawasaki Disease in Japan: Results from the Nationwide Survey in 2005-2006." J Epidemiol 18(4): 167-172.7. Park, Y., J. Han, et al. (2007). "Kawasaki Disease in Korea, 2003-2005." Pediatric Infectious Disease Journal 26(9): 821-823.8. Robert C. Holman, A. T. C., Ermias D. Belay, Claudia A. Steiner and Lawrence B. (2003). "Kawasaki Syndrome Hospitalizations in the United States, 1997 and 2000." Pediatrics 112(3): 495-501.
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