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Kawasaki disease

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  • 1. Prof DR Dr Arijanto Harsono SpAK
  • 2. 1967 Kawasaki Tomisaku reported50 cases with febrile,servical lymphadenopathy,conjunctival redness,red tongue, cracked lips,erythema, and swollen handsand feet exfoliation followedPENDAHULUANKAWASAKI DISEASE (KD)Previously called: MUCOCUTANEOUS LYMPH NODE SYNDROMEVasculitis DISEASES AFTER THE SECOND MOST HENOCHSchönlein Purpura2Prof DR Dr Ariyanto Harsono SpA(K)
  • 3. ETIOLOGY/PATHOGENESISCoronary vasculitis and medium blood vesselsSuper-Antigen exposure  HSP65Activation of the immune systemActivation of endothelial cells and monocytesActivation of T cells and B cellsIncreased inflammatory cytokines3Prof DR Dr Ariyanto Harsono SpA(K)
  • 4. 4Prof DR Dr Ariyanto Harsono SpA(K)
  • 5. Prof DR Dr Ariyanto Harsono SpA(K)
  • 6. ETIOLOGY/PATHOGENESIS...6Prof DR Dr Ariyanto HarsonoSpA(K)
  • 7. ETIOLOGY/PATHOGENESIS...7Prof DR Dr Ariyanto Harsono SpA(K)
  • 8. Clinical Manifestations8Prof DR Dr Ariyanto Harsono SpA(K)
  • 9. Clinical Manifestations...9Prof DR Dr Ariyanto Harsono SpA(K)
  • 10. ClNICAL MANIFESTATION...10Prof DR Dr Ariyanto Harsono SpA(K)
  • 11. 11Prof DR Dr Ariyanto Harsono SpA(K)ClNICAL MANIFESTATION...
  • 12. CLINICAL MANIFESTATION...12Prof DR Dr Ariyanto Harsono SpA(K)
  • 13. CLINICAL MANIFESTATIONS...13Prof DR Dr Ariyanto HarsonoSpA(K)
  • 14. Prof DR Dr Ariyanto HarsonoSpA(K)14CLINICAL MANIFESTATIONS...Prof DR Dr Ariyanto Harsono SpA(K)
  • 15. CLINICAL MANIFESTATIONS...15Prof DR Dr Ariyanto Harsono SpA(K)
  • 16. CLINICAL MANIFESTATIONS16Prof DR Dr Ariyanto Harsono SpA(K)CLINICAL MANIFESTATIONS……
  • 17. CLINICAL MANIFESTATIONS...17Prof DR Dr Ariyanto Harsono SpA(K)
  • 18. 18Prof DR Dr Ariyanto Harsono SpA(K)CLINICAL MANIFESTATIONS……
  • 19. CLINICAL MANIFESTATIONS …19Prof DR Dr Ariyanto Harsono SpA(K)
  • 20. 20Prof DR Dr Ariyanto Harsono SpA(K)CLINICAL MANIFESTATIONS……
  • 21. 21Prof DR Dr Ariyanto Harsono SpA(K)CLINICAL MANIFESTATIONS……..
  • 22. Prof DR Dr Ariyanto HarsonoSpA(K)22CLINICAL MANIFESTATIONS……..CLINICAL MANIFESTATIONS…….
  • 23. 23Prof DR Dr Ariyanto Harsono SpA(K)CLINICAL MANIFESTATIONS…….
  • 24. 24Prof DR Dr Ariyanto Harsono SpA(K)CLINICAL MANIFESTATIONS…….
  • 25. MANIFESTASI KLINIS...25Prof DR Dr Ariyanto Harsono SpA(K)CLINICAL MANIFESTATIONS…….
  • 26. Unusual manifestationProf DR Dr Ariyanto Harsono SpA(K) 26Angus’ Rash
  • 27. Unusual manifestation…..Prof DR Dr Ariyanto HarsonoSpA(K)27Prof DR Dr Ariyanto Harsono SpA(K)
  • 28. Unusual manifestation…..Prof DR Dr Ariyanto HarsonoSpA(K)28Beau’s line
  • 29. Unusual manifestation…..29Prof DR Dr Ariyanto HarsonoSpA(K)
  • 30. DIAGNOSISDiagnosis Criteria:30Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorph EksantemCervical adenopathyTypical KD: Fever 5days with 4 or more ofthe following criteria
  • 31. DIAGNOSISKriteria Diagnosis:Typical KD: Demam 5 hari ataulebih disertai 4 dari kriteriadibawah ini31Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorphEksantemCervical adenopathy
  • 32. DIAGNOSISKriteria Diagnosis:Typical KD: Demam 5 hari ataulebih disertai 4 dari kriteriadibawah ini32Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorph ExanthemCervical adenopathy
  • 33. DIAGNOSISKriteria Diagnosis:Typical KD: Demam 5 hari ataulebih disertai 4 dari kriteriadibawah ini33Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorph ExanthemCervical adenopathy
  • 34. DIAGNOSISKriteria Diagnosis:Typical KD: Demam 5 hari ataulebih disertai 4 dari kriteriadibawah ini34Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorph ExanthemCervical adenopathy
  • 35. DIAGNOSISKriteria Diagnosis:Typical KD: Demam 5 hari ataulebih disertai 4 dari kriteriadibawah ini35Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorph ExanthemCervical adenopathy
  • 36. DIAGNOSISKriteria Diagnosis:Typical KD: Demam 5 hari ataulebih disertai 4 dari kriteriadibawah ini36Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorph ExanthemCervical adenopathy
  • 37. DIAGNOSISKriteria Diagnosis:Typical KD: Demam 5 hari ataulebih disertai 4 dari kriteriadibawah ini37Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorphEksanthemCervical adenopathy
  • 38. DIAGNOSISKriteria Diagnosis:Typical KD: Demam 5 hari ataulebih disertai 4 dari kriteriadibawah ini38Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorphEksanthemCervical adenopathy
  • 39. DIAGNOSISKriteria Diagnosis:39Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorphEksanthemCervical adenopathy
  • 40. DIAGNOSISDiagnosisCriteria:40Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesPolymorphEksanthemCervical adenopathy
  • 41. DIAGNOSISDiagnosis Ceriteria:41Bilateral non-purulentconjunctivitisChanges in lips andoral cavityPeripheral limbabnormalitiesEksantempolymorphCervical adenopathy
  • 42. Atypical KD:Fever 5 days or more criteria +3 or lessFever 5 + days or more coronaryabnormalities42Prof DR Dr Ariyanto Harsono SpA(K)
  • 43. • No history of sorethroat or evidence oftonsillar exudate• Streptococcalserology: negative..• Scarlet fever isthought to be unlikely.Prof DR Dr Ariyanto HarsonoSpA(K)43
  • 44. Laboratry ExaminationsProf DR Dr Ariyanto Harsono SpA(K) 44LEDCRPLeukocyteNeutrophilThrombocyteCholesterolHDLTrigliseridPlasma Cell IgAPerinuclear anti-neutrophiliccytoplasmic antibodies (P-ANCA): +
  • 45. 45Prof DR Dr Ariyanto Harsono SpA(K)
  • 46. Pathology examination...Intima and surrounding tissue necrosis. Areas ofnecrosisshowed fibrinoid change and basophilik.Inflammatory cell infiltrates-2 and the rest of thecore seen in areas of necrosis.Picture of early necrosis; smooth muscleshowed cytoplasmic acidofiliaCore looks picnotic (chromatincondensation).Adventisia contained infiltrates ofinflammatory cells.46Prof DR Dr Ariyanto Harsono SpA(K)
  • 47. Arteriole: fibrinoid necrosis (leukocytoclastic vasculitis):Note the pink staining material (fibrinoid necrosis) in multifocal areas of thethickened wall of the venule. The material represents protein derived from theplasma that has deposited in the vessel wall owing to an increase in vesselpermeability from the inflammatory process.• It is called fibrinoid because it lookslike fibrin in a clot but it is really protein.Small vessel vasculitis is usually due toimmune complex (IC) disease (type IIIhypersensitivity). ICs are deposited inthe vessel wall and then activate thecomplement system. C5a, achemotactic factor, attracts neutrophils(only a few are visible at around 7o’clock).Prof DR Dr Ariyanto HarsonoSpA(K)47
  • 48. MANAGEMNTProf DR Dr Ariyanto Harsono SpA(K)48IVIG: Should be given within thefirst 10 days after thediagnosis is established Dosage: 2g/Kg single dose Children: 1g/kg BW shouldbe given "Single infusion" in8-12 hours. If you alreadyhave heart problems givenin divided doses 3-4 days
  • 49. Prof DR Dr Ariyanto HarsonoSpA(K)49
  • 50. Management…Patients who are refractory to IVIG: Pulse methylprednisolone 0.5-2 mg / kg bwAspirindose:50-80 mg / kg bw in the acute inflammatory phase3-5 mg / kg bw after fever resolved and platelets increased,maintained until cardiac abnormalities improvedDipridamole: In patients who are intolerant to aspirinDose: 2-3 mg / kg bw50Prof DR Dr Ariyanto Harsono SpA(K)
  • 51. Prof DR Dr Ariyanto HarsonoSpA(K)51
  • 52. PROGNOSISWere largely complete recoveryLong-term observation of patients with aneurysmsremaining 50% improved cardiac abnormalitiesMortality 1970: 1-2%; 1990: 0.4% due to the blockageof coronary / other cardiac abnormalities95% of deaths occurred after 6 months because ofinfection, the rest after 10 years52Prof DR Dr Ariyanto Harsono SpA(K)
  • 53. ProfilaksisDentists should remain alertto features of the acutedisease, and in patients witha history of Kawasakidisease, be aware of thepossibility of recurrence andof heart valve defectsrequiring antibioticprophylaxis prior to relevantdental treatment.Prof DR Dr Ariyanto Harsono SpA(K) 53
  • 54. Prof Kawasaki Karlee54)
  • 55. Prof DR Dr Ariyanto HarsonoSpA(K)55Prof Takashimura
  • 56. 56Prof DR Dr Ariyanto HarsonoSpA(K)

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