Kawasaki Disease

1,412 views

Published on

Overview of Kawasaki Disease

  • Be the first to comment

  • Be the first to like this

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.

×