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Kawasaki disease

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KAWASAKI DISEASE
History of Kawasaki disease
Epidemiology and etiology
Presentation and diagnosis
Treatment
Chronic cardiovascular manifestations
Follow up of patients
Questions in the chronic management

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Kawasaki disease

  1. 1. KAWASAKI DISEASEPresented byPresented byDr. PANKAJ YADAVDr. PANKAJ YADAVdrpankajyadav05@gmail.comdrpankajyadav05@gmail.comdrpankajyadav05@gmail.com
  2. 2. Kawasaki Disease• History of Kawasaki disease• Epidemiology and etiology• Presentation and diagnosis• Treatment• Chronic cardiovascular manifestations• Follow up of patients• Questions in the chronic managementdrpankajyadav05@gmail.com
  3. 3. Kawasaki Disease(MucocutaneousLymph Node Syndrome)“A self-limited vasculitis of unknownetiology that predominantly affects childrenyounger than 5 years. It is now the mostcommon cause of acquired heart disease inchildren in the United States and Japan.”Jane Burns, MD**Burns, J. Adv. Pediatr. 48:157. 2001.*Burns, J. Adv. Pediatr. 48:157. 2001.drpankajyadav05@gmail.com
  4. 4. History of Kawasaki Disease• Original case observed by Kawasaki January 1961– 4 y.o. boy, “diagnosis unknown”• CA thrombosis 1strecognized 1965 on autopsy ofchild prev. dx’d w/MCOS• First Japanese report of 50 cases, 1967• First English language report from Dr. Kawasaki1974, simultaneously recognized in Hawaiidrpankajyadav05@gmail.com
  5. 5. What is Kawasaki Disease?• Idiopathic multisystem diseasecharacterized by vasculitis of small &medium blood vessels, includingcoronary arteriesdrpankajyadav05@gmail.com
  6. 6. Epidemiology• Median age of affected children = 2.3 years• 80% of cases in children < 4 yrs, 5% ofcases in children > 10 yrs• Males:females = 1.5-1.7:1• Recurs in 3%• Positive family history in 1% but 13% riskof occurrence in twins.drpankajyadav05@gmail.com
  7. 7. Epidemiology• Annual incidence of 4-15/100,000 childrenunder 5 years of age• More in Asian-Americans, African-Americans next most prevalent• Seasonal variation– More cases in winter and spring but occursthroughout the yeardrpankajyadav05@gmail.com
  8. 8. What is the Etiology of KawasakiDisease?drpankajyadav05@gmail.com
  9. 9. Etiology• Infectious agent most likely– Age-restricted susceptible population– Seasonal variation– Well-defined epidemics– Acute self-limited illness similar to knowninfections• No causative agent identified– Bacterial, retroviral, superantigenic bacterial toxin– Immunologic response triggered by one of severalmicrobial agentsdrpankajyadav05@gmail.com
  10. 10. New Haven Coronavirus• Identified a novel human coronavirus inrespiratory secretions from a 6-month-oldwith typical Kawasaki Disease• Subsequently isolated from 8/11 (72.7%) ofKawasaki patients & 1/22 (4.5%) matchedcontrols (p = 0.0015)• Suggests association between viral infection& Kawasaki diseaseEsper F, et . J Inf Dis. 2005; 191:499-502drpankajyadav05@gmail.com
  11. 11. Diagnostic Criteria• Fever for at least 5 days• At least 4 of the following 5 features:1. Changes in the extremities Edema, erythema, desquamation2. Polymorphous exanthem, usually truncal3. Conjunctival injection4. Erythema&/or fissuring of lips and oral cavity5. Cervical lymphadenopathy• Illness not explained by other known diseaseprocessModified from Centers for Disease Control. Kawasaki Disease. MMWR 29:61-63, 1980drpankajyadav05@gmail.com
  12. 12. Atypical or IncompleteKawasaki Disease• Present with < 4 of 5 diagnostic criteria• Compatible laboratory findings• Still develop coronary artery aneurysms• No other explanation for the illness• More common in children < 1 year of age• 2004 AHA guidelines offer new evaluationand treatment algorithmdrpankajyadav05@gmail.com
  13. 13. Differential Diagnosis• Infectious– Measles & Group A beta-hemolytic strep canclosely resemble KD– Bacterial: severe staph infections w/toxinrelease– Viral: adenovirus, enterovirus, EBV, roseoladrpankajyadav05@gmail.com
  14. 14. Differential Diagnosis• Infectious– Spirocheteal: Lyme disease, Leptospirosis– Parasitic: Toxoplasmosis– Rickettsial: Rocky Mountain spotted fever,Typhusdrpankajyadav05@gmail.com
  15. 15. Differential Diagnosis• Immunological/Allergic– JRA (systemic onset)– Atypical ARF– Hypersensitivity reactions– Stevens-Johnson syndrome• Toxins– Mercurydrpankajyadav05@gmail.com
  16. 16. Phases of Disease• Acute (1-2 weeks from onset)– Febrile, irritable, toxic appearing– Oral changes, rash, edema/erythema of feet• Subacute (2-8 weeks from onset)– Desquamation, may have persistent arthritis orarthralgias– Gradual improvement even without treatment• Convalescent (Months to years later)drpankajyadav05@gmail.com
  17. 17. Trager, J. D. N Engl J Med 333(21): 1391. 1995.drpankajyadav05@gmail.com
  18. 18. img0017.jpgdrpankajyadav05@gmail.com
  19. 19. drpankajyadav05@gmail.com
  20. 20. kawasaki_3_010520.jpgdrpankajyadav05@gmail.com
  21. 21. drpankajyadav05@gmail.com
  22. 22. Trager, J. D. N Engl J Med 333(21): 1391. 1995.drpankajyadav05@gmail.com
  23. 23. Han, R. CMAJ 162:807. 2000. drpankajyadav05@gmail.com
  24. 24. Kawasaki Disease:symptoms andsigns• Respiratory– Rhinorrhea, cough, pulmonary infiltrate• GI– Diarrhea, vomiting, abdominal pain, hydrops ofthe gallbladder, jaundice• Neurologic– Irritability, aseptic meningitis, facial palsy,hearing loss• Musculoskeletal– Myositis, arthralgia, arthritisdrpankajyadav05@gmail.com
  25. 25. Kawasaki Disease: Lab findings• Early– Leukocytosis– Left shift– Mild anemia– Thrombocytopenia/Thrombocytosis– Elevated ESR– Elevated CRP– Hypoalbuminemia– Elevated transaminases– Sterile pyuria• Late– Thrombocytosis– Elevated CRPdrpankajyadav05@gmail.com
  26. 26. Cardiovascular Manifestations ofAcute Kawasaki Disease• EKG changes– ArrhythmiasArrhythmias– Abnormal Q wavesAbnormal Q waves– Prolonged PR and/or QT intervalsProlonged PR and/or QT intervals– Low voltageLow voltage– ST-T–wave changes.ST-T–wave changes.• CXR–cardiomegalydrpankajyadav05@gmail.com
  27. 27. Cardiovascular Manifestations ofAcute Kawasaki Disease• None• Suggestive of myocarditis (50%)Suggestive of myocarditis (50%)– Tachycardia, murmur, gallop rhythmsTachycardia, murmur, gallop rhythms– Disproportionate to degree of fever & anemiaDisproportionate to degree of fever & anemia• Suggestive of pericarditisSuggestive of pericarditis– Present in 25% although symptoms are rarePresent in 25% although symptoms are rare– Distant heart tones, pericardial friction rub,Distant heart tones, pericardial friction rub,tamponadetamponadedrpankajyadav05@gmail.com
  28. 28. Role of Cardiology in the AcuteSetting• Usually just to document baseline coronaryartery status–not an emergency• If myocarditis suspected–an emergency• Can help diagnose “atypical” diseasedrpankajyadav05@gmail.com
  29. 29. Echocardiographic Findings• Myocarditis with dysfunction• Pericarditis with an effusion• Valvar insufficiency• Coronary arterial changesdrpankajyadav05@gmail.com
  30. 30. Coronary Arterial Changes• 15% to 25 % of untreated patientsdevelop coronary artery changes• 3-7% if treated in first 10 days of feverwith IVIG• Most commonly proximal, can be distal– Left main > LAD > Rightdrpankajyadav05@gmail.com
  31. 31. Coronary Arterial Changes• Vary in severity from echogenicity dueto thickening and edema orasymptomatic coronary artery ectasiato giant aneurysms• May lead to myocardial infarction,sudden death, or ischemic heart diseasedrpankajyadav05@gmail.com
  32. 32. Coronary Aneurysms• Size– Small = <5 mm diameter– Medium = 5-8 mm– Giant = ≥ 8 mm• Highest risk for sequelae• Shape– Saccular– Fusiformdrpankajyadav05@gmail.com
  33. 33. Coronary Aneurysms•• Patients most likely to develop aneurysms– Younger than 6 months, older than 8 years– Males– Fevers persist for greater than 14 days– Persistently elevated ESR– Thrombocytosis– Pts who manifest s/s of cardiac involvementdrpankajyadav05@gmail.com
  34. 34. Circulation 103(2):335-336. 2001.drpankajyadav05@gmail.com
  35. 35. Coronary Aneurysm• Approximately 50% of aneurysms resolve– Smaller size– Fusiform morphology– Female gender– Age less than 1 year• Giant aneurysms (>8mm) worst prognosisdrpankajyadav05@gmail.com
  36. 36. Cardiovascular Sequelae• 0.3-2% mortality rate due to cardiac disease– 10% from early myocarditis• Aneurysms may thrombose, cause MI/death• MI is principal cause of death in KD– 32% mortality– Most often in the first year– Majority while at rest/sleeping– About 1/3 asymptomaticdrpankajyadav05@gmail.com
  37. 37. Acute Kawasaki Disease:Treatment• IVIG: 2g/kg as one-time dose– Mechanism of action is unclear– Significant reduction in CAA in ptstreated with IVIG plus aspirin vs. aspirinalone (15-25%3-5%)– Efficacy unclear after day 10 of illnessdrpankajyadav05@gmail.com
  38. 38. Acute Kawasaki Disease:Treatment• IVIG– 70-90% defervesce & show symptomresolution within 2-3 days of treatment– Retreat those with failure of response to1stdose or recurrent symptoms  Up to2/3 respond to a second coursedrpankajyadav05@gmail.com
  39. 39. Acute Kawasaki Disease:Treatment• Aspirin– High dose (80-100 mg/kg/day) until afebrilex 48 hrs &/or decrease in acute phasereactants– Need high doses in acute phase due tomalabsorption of ASA– Dosage of ASA in acute phase does notseem to affect subsequent incidence of CAAdrpankajyadav05@gmail.com
  40. 40. Acute Kawasaki Disease:Treatment• Aspirin– Decrease to low dose (3-5 mg/kg/day) for 6-8weeks or until platelet levels normalize– No evidence f/effect on CAA when usedalone– Due to potential risk of Reye syndromeinstruct parents about symptoms of influenzaor varicelladrpankajyadav05@gmail.com
  41. 41. Acute Kawasaki Disease:Treatment• Aggressive support with diuretics &inotropes for some patients withmyocarditis• Antibiotics while excluding bacterialinfectiondrpankajyadav05@gmail.com
  42. 42. Acute Kawasaki Disease:Treatment• Conflicting data about steroids– Reports of higher incidence of aneurysms &more ischemic heart dz in pts w/aneurysms– Case report of KD refractory to IVIG butresponsive to high-dose steroids &cyclosporine.drpankajyadav05@gmail.com
  43. 43. Patient Follow-Up Categories• Five categories based on coronary arteriesfindings– 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 ormultiple smaller/complex aneurysms in sameCA, without obstruction– Coronary artery obstructiondrpankajyadav05@gmail.com
  44. 44. Management Categories• Pharmacologic therapy• Physical activity• Follow-up and diagnostic testing• Invasive testingdrpankajyadav05@gmail.com
  45. 45. I. No coronary changes at anystage 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 recommendeddrpankajyadav05@gmail.com
  46. 46. II. Transient CA ectasia, resolvedwithin 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 yrintervals• Invasive testing– None recommendeddrpankajyadav05@gmail.com
  47. 47. III. Single Small or Medium SizeAneurysm• 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 stresstest/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 ischemiadrpankajyadav05@gmail.com
  48. 48. IV. Aneurysms without Stenosis• Pharmacologic Therapy– Long-term antiplatelet tx & warfarin or LMWH• Physical Activity– Restrictions based on stress test/myocardial perfusionscan– 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 clinicallyindicated– Elective repeat in certain circumstancesdrpankajyadav05@gmail.com
  49. 49. V. Obstruction• Pharmacologic Therapy– Long-term low-dose ASA, ± warfarin or LMWH if giantaneurysm 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..drpankajyadav05@gmail.com
  50. 50. drpankajyadav05@gmail.com

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