KAWASAKI DISEASE

I. Definitions and pathophysiology

  1. aka "Mucocutaneous lymph node syndrome"
  2. A multisystem vasculitis
  3. Incidence limited to children

II. Clinical features

  1. Epidemiology
    1. 50% occurs before 2.5 y; 35% 2.5-5y, rare >10y
    2. Most common in kids of Asian or African descent
    3. Male > Female
    4. Fatal in 0.5% of cases, more likely to be fatal in boys and pts < 3yo
  2. Presents with fever, usually > 5d
  3. Usually tachycardic; may have mildly prolonged PR interval, TWI or flattening, and nonspecific ST changed.
  4. Labs: usually have high WBC w/left shift, high platelet counts, high ESR and CRP; sterile pyuria and albuminuria sometimes noted
  5. Can cause coronary vasculitis and aneurysms with fatal complications
  6. Other cardiac abnormalities reported: AR, MR, conduction system dysfunction, myocardial dysfunction, and myocardial fibrosis
  7. Diagnostic criteria--Need fever x > 5d & four of the following, w/o evidence of other cause for the sx. If coronary aneurysms are documented, only 3 of the following are required for dx:
  1. Conjunctival injection (bilateral, nonexudative)
  2. Oropharyngeal lesions (red or cracked lips, strawberry tongue, or injected mucosae)
  3. Erythematous rash (usually "blotchy")
  4. Erythema, edema, or desquamation of hands or feet
  5. Cervical lymphadenopathy (50-75% of pts)

III. Treatment

  1. ASA--continue until all lab abnormalities are resolved
  2. Intravenous Immune Globulin (IVIG)--helps reduce symptoms & risk of coronary aneurysms, but can cause fever, chills, hypotension, and rarely, hemolytic anemia
  3. Corticosteroids
    1. 39 pts with Kawasaki's randomized to methylprednisolone 30mg/kg IV; all pts also received ASA and were randomized to receive IVIG. Compared with the ASA-IVIG-alone group, the ASA-IVIG-steroid group had sig. shorter mean duration of fever (1.0 vs. 2.4d) and mean duration of hospital stay (1.9 vs. 3.3d) (J. Peds. 142:611, 2003--abst)
    2. In a meta-analysis of 8 randomized studies of corticosteroids vs. placebo in pts with Kawasaki's disease, incidence of coronary artery aneurysms was sig. lower in corticosteroid recipients (OR 0.55); only 3 of these studies included IVIG as part of standard therapy, though steroids were found to be beneficial in an analysis of just those three studies as well (Peds. 116:989, 2005--JW)
    3. In a study in 178 pts with Kawasaki disease, all of whom were treated with IVIG, randomized to prednisolone 2mg/kg/d vs. placebo until temperature and CRP were normal, there was no sig. diff. in prevalence of coronary artery abnormalities at 1mo, though prednisolone group had sig. faster resolution of fever and high CRP (J. Peds. 149:336, 2006--JW)
    4. In a study in in 199 children with Kawasaki disease and fever for 10d randomized to methylprednisolone 30mg/kg IV x 1 vs. placebo (all received IVIG + ASA), there were no sig. diffs. at 1wk or 5wks in various measures of coronary disease though IV steroid recipients had shorter initial hospitalization stays (NEJM 356:659, 2007--JW)
  4. Follow-up echocardiography recommended by AHA (as of 2004) 6-8wks after initial presentation and again 6-12mos later; however, the latter was not likely to show abnormalities in pts whose first f/u echo was normal in one retrospective study (Am. J. Cardiol. 88:328, 2001--AFP)

(Source: West. J. Med. 168:23, 1998--AFP and others...)