Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.

Kawasaki disease


Published on

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

Published in: Health & Medicine
  • Be the first to comment

Kawasaki disease

  1. 1. KAWASAKI DISEASEPresented byPresented byDr. PANKAJ YADAVDr. PANKAJ
  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
  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.
  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
  5. 5. What is Kawasaki Disease?• Idiopathic multisystem diseasecharacterized by vasculitis of small &medium blood vessels, includingcoronary
  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
  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
  8. 8. What is the Etiology of KawasakiDisease?
  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
  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;
  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,
  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
  13. 13. Differential Diagnosis• Infectious– Measles & Group A beta-hemolytic strep canclosely resemble KD– Bacterial: severe staph infections w/toxinrelease– Viral: adenovirus, enterovirus, EBV,
  14. 14. Differential Diagnosis• Infectious– Spirocheteal: Lyme disease, Leptospirosis– Parasitic: Toxoplasmosis– Rickettsial: Rocky Mountain spotted fever,
  15. 15. Differential Diagnosis• Immunological/Allergic– JRA (systemic onset)– Atypical ARF– Hypersensitivity reactions– Stevens-Johnson syndrome• Toxins–
  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)
  17. 17. Trager, J. D. N Engl J Med 333(21): 1391.
  18. 18.
  19. 19.
  20. 20.
  21. 21.
  22. 22. Trager, J. D. N Engl J Med 333(21): 1391.
  23. 23. Han, R. CMAJ 162:807. 2000.
  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,
  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
  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–
  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,
  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”
  29. 29. Echocardiographic Findings• Myocarditis with dysfunction• Pericarditis with an effusion• Valvar insufficiency• Coronary arterial
  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 >
  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
  32. 32. Coronary Aneurysms• Size– Small = <5 mm diameter– Medium = 5-8 mm– Giant = ≥ 8 mm• Highest risk for sequelae• Shape– Saccular–
  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
  34. 34. Circulation 103(2):335-336.
  35. 35. Coronary Aneurysm• Approximately 50% of aneurysms resolve– Smaller size– Fusiform morphology– Female gender– Age less than 1 year• Giant aneurysms (>8mm) worst
  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
  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
  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
  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
  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
  41. 41. Acute Kawasaki Disease:Treatment• Aggressive support with diuretics &inotropes for some patients withmyocarditis• Antibiotics while excluding
  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 &
  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
  44. 44. Management Categories• Pharmacologic therapy• Physical activity• Follow-up and diagnostic testing• Invasive
  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
  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
  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
  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
  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
  50. 50.