Kawasaki Disease

DEFINITION
1. also known as
  1. mucocutaneous lymph node syndrome
2. is a disease
  1. blood vessels
    1. through the body
      1. become inflamed
EPIDEMIOLOGY
1. vasculitis
  1. rare in children
  2. incidence
    1. 10 and 50 / 100.000
  3. < 5% of referrals to pediatric rheumatologist
    1. 2 common types deleted
      1. < 1%
        of chronic inflammatory disorders in childhood
  4. 2 common types
    1. kawasaki disease
      1. similar %
        have evidence of infection
        bacterial
        viral
        systemical
        local
      2. predominant children
        < 2 years
        rare in late childhood
    2. henoch schonlein (HSP)
      1. 1/3 of cases HSP
        after Streptococcal infection
  5. posible pathogenesis
    1. suggest a close connection
      1. transmisible agent
2. occurs regularly in 1980
  1. average of patients
    1. age decreased
    2. % of girls increased
3. decades of attempts to prove
  1. viruses
  2. bacterial toxins
  3. specific pathogens
    1. causes most cases
      1. not been subestimated
RISK FACTOR
1. most type of vasculitis
  1. not known
    1. rick factors are equally hidden
2. infections
3. inmunization
4. genetic factors
  1. inmunomoduladory
  2. haplotypic
  3. vascular genes
    1. do not explain phenomena
5. monogenic diseases
  1. vasculopathy
    1. associted with
      1. mutations in adenosine deaminase 2
PATHOPHYSIOLOGY
1. pathology characteristics
  1. primary target of inflammation
    1. medium-sized muscular arteries
  2. coronary arteries
    1. most affected
    2. most likely to suffer
      1. chronic damage
    3. neutrophils
      1. invade
        vessels walls
      2. neutrophil infiltrate
        evident in the arterial wall
        first 2 week of disease
2. immunologic response
  1. gene expression studies
    1. DNA microarrays
      1. from acute - phase whole blood
        confirm increased relative abundance with neutrophils
        adrenomodulin
        grancalcin
        granulin
        decreased NK
        decreased CD8 + lymphocytes
CLINICAL FEATURES
1. differ from chronic vaasculophaties
2. characteristics of any type of vasculitis
  1. fever
  2. malaise
  3. fatigue
  4. failure to thrive
  5. increased levels
    1. acute - phase reactant
3. progressions immits viral infections
  1. rash
    1. eritema
    2. desquamation
  2. mucosal inflammation
  3. extremity changes
4. non - exudative conjuntivitis
5. cracked
6. red lips
7. strawberry tongue
8. beau lines (nails)
9. arthritis
  1. commonly affects
    1. lower extremities
10. small joint polyarthritis
11. cervical lymphoadenopaty
LABORATORY FINDINGS
1. systemic inflammation
  1. PCR
  2. erytrocyte sedimentation rate
  3. Alpha 1 antitripsine
  4. leukocytosis
  5. increased platelets levels
2. normonytic,normochromic anemia
3. Hb concentration
  1. more than 2 DE
4. urianalysis
  1. white blood cells
5. liver enzymes
  1. increased transminase levels
  2. mild hyperbilirrubinemia
  3. obstructive jaundice
    1. from hydrops of gallbladder
6. cerebrospinal fluid
  1. pleocythosis
DIFFERENTIAL DIAGNOTICS
1. mucocutaneos inflammation
  1. measles
  2. echovirus
  3. adenovirus
2. toxin mediated illness
  1. mediated by B - hemolytic streptococcus
3. rocky mountain fever and leptospirosis
4. steven - johnson syndrome
  1. drug reaction
5. mercury hipersensitive reaction
6. PAN
7. rheumatoid arthritis
MANAGMENT
1. IVIG
  1. for 10 days
    1. reduces incidence of
      1. coronary artery aneurysm
        by more than 70%
2. aspirin
  1. antiinflammatory and antithrombotic effects
  2. initial dose 100 mg/kg/d
  3. once fever is solved
    1. 3 to 5 mg/kg/d

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