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A patient

A patient. 3 year-old boy, previously well 8 days of rash 7 days fever Treated for scarlet fever, no response Eyes red Hands & feet red & swollen Lips red & swollen Desquamating rash over groin. A picture. Starting off the new year right, with Kawasaki Disease Dave Rupar, MD

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A patient

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  1. A patient • 3 year-old boy, previously well • 8 days of rash • 7 days fever • Treated for scarlet fever, no response • Eyes red • Hands & feet red & swollen • Lips red & swollen • Desquamating rash over groin

  2. A picture

  3. Starting off the new year right, with Kawasaki Disease Dave Rupar, MD 3 January 2012

  4. Kawasaki Disease: Goals for today • Clinical & laboratory features • Etiology and pathogenesis • Cardiac complications • Treatment • Difficult situations

  5. Kawasaki Disease • Systemic vasculitis of unknown etiology which attacks young children, affecting medium to large blood vessels • Fever for five days plus 4/5: • Extremity changes • Polymorphous exanthem • Bilateral non-purulent bulbar conjunctivitis • Changes of lips and oral cavity • Cervical adenopathy • No other etiology

  6. 1871: London, Dr. Gee: “scarlatinal dropsy”, KD: some history

  7. 1871: “scarlatinal dropsy” 1967: Tomisaku Kawasaki reports 50 cases of “Pediatric acute febrile MLNS KD: some history Kawasaki, T. Japan J Allergy 1967:16:178, from PIDJ 2002; 21:1

  8. 1871: “scarlatinal dropsy” 1967: publication 1970: Cardiac complications recognized KD: some history Kawasaki, T. Japan J Allergy 1967:16:178, from PIDJ 2002; 21:1

  9. 1871: scarlatinal dropsy 1967: publication 1970: complications 1986: IVIG works KD: some history

  10. KD: Epidemiology • Y2K ~4000 hospitalization in US • Asian/PI: 32/100,000 <5 yo • Black: 17/100,000 • Hispanic: 11/100,000 • White: 9/100,000 • Boys>Girls 1.5:1

  11. KD: Epidemiology Pediatrics 2002;109:e87 6442 children hospitalized in the US, 1988-97

  12. Acute onset, self-limited Young children, but rare <3 months Recurrence rare Seasonal Outbreaks Increased risk in families ID consults Sounds like an infection…

  13. Acute onset, self-limited Young children, but rare <3 months Recurrence rare Seasonal Outbreaks Increased risk in families ID consults Racial predilection persists around the world No strong evidence of person to person spread Relatively low in families Failure to identify organism No it doesn’t!

  14. GAS S. aureus “Super-antigens” Mycoplasma Chlamydia Adenovirus CMV Coronavirus EBV HPV-B19 HHV-6, -8 Measles Retroviruses “Novel viruses” Microorganisms tried and found wanting

  15. Rug shampoo Dust mites Body of water Toxins (Hg?) Environmental factors?

  16. Population risks constant Slight increased risk in siblings (~2%), identical twins (~13%) 2nd generation cases Many putative genetic markers Genetic factors?

  17. KD: Putting it together? • Genetic predisposition • + right age • + infection • + environmental factors • = immune (over) activation

  18. KD: Putting it together? • Genetic predisposition • + right age • + infection • + environmental factors • = immune overactivation

  19. Fever for 5 days Plus 4/5 KD: Clinical features

  20. Fever for 5 days Plus 4/5 Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features

  21. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features Red, swollen hands & feet

  22. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features Periungual desquamation

  23. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features Beau’s lines

  24. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features

  25. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features

  26. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features Perineal desquamation

  27. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features Clinical Infect Dis 1999;28:169

  28. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features

  29. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features Strawberry tongue

  30. Extremity changes Polymorphous rash Conjuctivitis Oral mucosal changes Adenopathy KD: Clinical features Unilateral cervical node

  31. A disease of protean manifestations • Cardiovascular* • Musculoskeletal-arthralgias, arthritis • Gastrointestinal- abd pain, hepatitis, GB • CNS- irritability, meningitis, facial palsy • GU- meatitis • Eye- uveitis

  32. KD: Clinical features 110 children with Kawasaki Disease. Wang, et al. Pediatr Infect Dis J 2005;24:998

  33. GAS- scarlet fever Toxic shock syndrome Staph scalded skin Drug rash/SJS JRA (Still’s disease) Adenitis RMSF Leptospirosis DDx: fever and a rash

  34. Adenovirus Measles EBV Enterovirus Acrodynia (Hg) DDx: fever and a rash

  35. KD is NOT a laboratory diagnosis • Help confirm clinical diagnosis • Rule out other diseases (cultures, serum) • Incomplete or difficult case

  36. KD is NOT a laboratory diagnosis • Leukocytosis, neutrophilia • Thrombocytosis (late) • Anemia • Elevated acute phase reactants • Sterile pyuria • Hypoalbuminemia, hyponatremia • Elevated transaminases, GGT • CSF pleocytosis

  37. Cardiac complications • 20-25% of untreated patients (infants, older, atypical) • Echocardiogram at Dx, 2 & 6 weeks • Coronary abnormalities (CAA) • Arteritis • Ectasia • Aneurysms • Myocarditis very common, usually subclinical • Pericardial effusions

  38. Usually after 10 days Prox LAD, Prox RCA Most resolve Rupture is rare Reports of MI Predisposed to ASCD? Cardiac complications- CAA

  39. KD: treatment • Why? • Reduce inflammation • Decrease cardiac complications (2-4%) • Who? • Everyone who meets criteria • Atypical (more later) • When? • Day 5-10, day 4 if undeniable • How? • IVIG • ASA • Steroids? • Other (infliximab, etanercept)?

  40. KD: treatment • Appropriate treatment greatly reduces coronary risk • Most patients respond quickly • Better late than never • 2 gm/kg as single dose is standard • ASA: (High dose, then low dose) is much less important

  41. Another patient • 3 year old girl with classic KD • Treated on day 10 • Seemed better, went home • Back on day 13 with fever • What happened?

  42. Treatment non-responders • Persistent fever (>72 hours) in 8-23% • Failure to respond may be significant risk factor for complications • Alternatives: • Repeat IVIG 2/kg (once or twice?) • Steroids : methylprednisolone 30 mg/kg for 1-3 days? • Others: infliximab?

  43. Patient #3 • 5 month-old white boy • Fever for 9 days • Mild URI • Irritable • Bilateral conjunctivitis, non-purulent • No mucosal, extremity changes • Non-specific adenopathy • Rash on arms

  44. Let’s look at the lab work • WBC 17 with 20 bands; Hgb 10, Plt 700K • ESR 90 • Mild increase in AST & ALT • 3+ leukocytes in urine • CSF 15 wbc, normal protein & glucose • Does he have Kawasaki disease?

  45. Patient #3 • 5 month-old white boy • Fever for 9 days • Mild URI • Irritable • Bilateral conjunctivitis, non-purulent • No mucosal, extremity changes • Non-specific adenopathy • Rash on arms

  46. The punch line is… • Coronary dilitation • Responded clinicaly to IVIG • Desquamated

  47. Diagnosis isn’t always easy Have Kawasaki Disease Meet clinical criteria

  48. KD: Dealing with atypical cases • Fever and 2 or 3 criteria • FWS >7 days in infants < 6months? • Is the pattern consistent? • Elevated acute phase reactants? • Other supplemental labs • Continual reassessment • Get help • Echo and treat if dx is likely • Refer to algorithm Pediatrics 2004; 114:1708

  49. Kawasaki Disease: The End

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