Kawasaki Disease aka Mucocutaneous Lymph Node syndrome
(CHLA Kawasaki Map),
Evaluation flowsheet of atypical disease

 Table 1. Principal Diagnostic Criteria for Kawasaki Disease 

Fever x 5 days + 4 of the following 5. Fever is abrupt onset, high-spiking, remittent. Duration is 11 days without treatment, resolves within 2 days with treatment.

Table 2. Associated Clinical Features of Kawasaki Dz

Extreme irritability
Arthralgia, arthritis
Aseptic meningitis
Cardiac disease
Hepatic dysfunction
Gallbladder hydrops
Otitis media, pneumonia

Neutrophilia with immature forms
·          Elevated ESR, CRP
Thrombocytosis (nl in week 1, >1 million in week 2-3), normocytic anemia
Sterile pyuria (week 1: 1/3 of pt have sterile pyuria of urethral origin, may be intermittent)
Elevated levels of serum transaminases. ANA neg. RF neg.

 …a generalized vasculitis of unknown cause that is a leading cause of acquired heart disease in children who live in the United States.
Consider Kawasaki in febrile children with cervical adenitis, unresponsive to ABX, without alternative ABX.

 1 . Epidemiology. Approximately 80% of patients are younger than 4 years of age; median age 2 y.o.; the occurrence of this disease after 8 years of age is unusual. Less than 2-3% of patients have recurrences, siblings 2%. Children of Asian ancestry experience the highest incidence. The greatest number of cases occur in the winter and spring, and epidemics have been reported. Person‑to‑person transmission has not been documented. Rare in age < 3 mos, due to protective maternal AB.

 2. Etiology. The cause remains unknown. Clinical and epidemiologic characteristics suggest an infectious agent. Infection may lead to an immune‑mediated disease in certain genetically predisposed children. ?ubiquitous infectious agent hitting genetically predisposed individuals (adults immune, young infants protected by maternal ab)

3. Clinical features. The diagnosis is established by the presence of fever and at least four of the five other principal clinical criteria when no other explanations for the illness exist (Table 1). Atypical cases that do not fulfill clinical criteria but are associated with risk of coronary artery aneurysms have been described. Atypical disease is more common under 1 year of age. Other clinical and laboratory findings associated with Kawasaki disease are presented in Table 2. Young adults may present with ischemic heart disease, as sequela of unrecognized disease in childhood.

    Clinical Phases

  1. 1-2 weeks - acute febrile phase (fever, conjuctivae, oral, hand/foot, rash, LAD, meningits, diarrhea, myocarditis common at this time +/- pericardial effusion. Coronary arteritis may be active, but aneurysms are generally not visible by echo

  2. subacute phase: from fever onset to 1 month: fever, rash, LAD resolve. Thrombocytosis, digit desquam, coronary artery aneurysms usually develop at this time

  3. convalescent stage, ends by 6-8 weeks: clinical signs are gone. ESR still abnormal.

4. Diagnosis and differential diagnosis. No specific diagnostic test is available. The differential diagnosis includes scarlet fever (responds rapidly to PCN, no elevated ESR, CRP, GABHS+ strep throat (although pt may also be a carrier)), staphylococcal scalded skin syndrome,toxic shock syndrome, Rocky Mountain spotted fever, leptospirosis, Stevens‑Johnson syndrome, drug reactions (periorbital edema), and juvenile rheumatoid arthritis, measles (exudative conjunctivitis, Koplik, severe cough, rash is behind ears, confluent as fades, residual brownish hue, also has swelling of hands, feet. Get measles IgM titer.). Also in ddx, rare: Macrophage Activation Syndrome

Labs are not diagnostic. Helpful studies include: ESR, CRP, Chem panel, CBC/diff, UA

Common pitfalls in diagnosis

Incomplete KD (formally called “atypical KD”)

Evaluation flowsheet of atypical disease

Red Flags that this may not be Kawasaki Disease…

Kawasaki dz vs Viral (very general): In viral infection, ESR/CRP often normal, platelets normal by day 7 (in Kawasaki >450), WBC nl with lymphocyte dominance (vs elev WBC and left shift in Kawasaki's).

5. Therapy. High doses of aspirin are given during the acute phase to counter inflammation. After the child is afebrile, low doses of aspirin are given for their antithrombotic effect. Intravenous gamma globulin administered within the first 10 days of illness can significantly  reduce the risk of coronary artery abnormalities. 

  1. Aspirin

  2. IVIG

  3. Long term treatment

    1. None needed if (-) ECHO at 6-8 week f/u visit

    2. Only antiplatelet therapy if solitary small aneurysm

    3. Add warfarin if have large or multiple aneurysms for antithrombotic effect

    4. Add B-blocker if have coronary artery occlusion and at higher risk of MI

6. Complications. Occasional recurrences (3%), or cases in siblings (2%). Kawasaki disease is usually self‑limited, but coronary artery abnormalities including aneurysms, develop in 15-20% of untreated patients. Coronary dilatation is first detectable by day 10, usually within 4 weeks of onset; aneurysms between day 19-25 (weeks). Fate of coronary aneurysms: 50% resolve angiographically 5-18 mos. If persistent 50% get smaller, 1/3 have no aneurysms. Fatalities are rare and occur with myocardial infarctions secondary to thrombosis within a coronary artery aneurysm, during week 3-4. Young infants are at greatest risk of cardiac disease. You may also see aneurysms of the middle sized arteries, most notably the axillary and iliac arteries, but also celiac, femoral, renal, brachial.May also see pancarditis, poor LV function, mitral valve regurgitation.


Risk Stratification
1.No coronary artery changes
2.Transient ectasia for only 6-8 weeks
3.One small-medium aneurysm
4.One or more large aneurysms
5.Coronary artery obstruction

Things to think about in KD kids who got IVIG and Aspirin

Take home points –areas of focus previously on Boards

Review questions

  1. 5 month old with fever x7 days, of unknown etiology, no other symptom.

  2. 2 y/o in day #12 of fever with peeling extremities and 2cm unilateral cervical LN. Had h/o resolved conjuncitivitis and red lips.

  3. 3 y/o with h/o recent febrile illness that lasted 10 days, now 2 days later afebrile. Had rash, and now has peeling extremities

  4. 4.Name the 7 “Red Flags” that point the diagnosis away from Kawasaki Disease.

  5. Name the order in which the criteria appear.

  6. Name 3 additional labs besides ESR/ CRP that can influence the decision to treat KD in patients who have incomplete disease, high ESR/ CRP, but normal ECHO.


  1. Check ESP/ CRP. If + check ECHO

  2. Do ECHO. IVIG since still febrile. High dose aspirin until AF for 48-72 hours, then change to low dose.

  3. ESR, CRP, platelets (all should be elevated). ECHO. If both are positive (pt has elevated ESR/ CRP and has CAA), then give IVIG. Low dose aspirin since now afebrile for 48 hours.

NMS Pediatrics
Pediatric Annals 34(1) Jan 2005
CHLA Board review 2005 (AAP/ AHA Endorsed Clinical Report “Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease for Health Professionals From the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association” Pediatrics, Vol. 114 No.6 December 2004)

Commentary by Mobeen H. Rathore MD, FAAP
Pediatric Infectious Diseases and Immunology, University of Florida Health Science Center, Jacksonville, FL

Prior studies of the use of corticosteroids for KD have produced conflicting results.1-5 They have, however, been flawed by retrospective, non-randomized, non-blinded design and non-standard IVIG dosing. The current investigation is the first randomized (although not blinded) study of corticosteroid use in combination with the standard treatment of KD (IVIG/ASA). Although the number of subjects was small, detrimental effects were not noted whereas benefits of corticosteroids, including shorter hospital stay and duration of fever, were seen. The clinical significance of the lower mean temperature during the first day of IVMP therapy and lower acute phase reactants (ESR, CRP) 6 weeks after IVMP therapy, however, require further clarification. No differences in coronary dimensions were noted between the 2 treatment groups at the 6-week visit. The accompanying editorial cautions against the use of corticosteroids as a routine management strategy and supports a second dose of IVIG as the preferential rescue therapy.6 This seems to be the prudent strategy until large, multicenter, prospective, randomized, blinded studies confirm or refute the benefit of corticosteroid use in the management of KD. Until that time, corticosteroids should be used as adjunctive therapy of unusual and severe cases of KD,7 and not routinely used for initial management


  1. Kato H, et al. Pediatrics. 1979;63:175-179.
  2. Wright DA, et al. J Pediatr. 1996;128:146-149.
  3. Shinohara M, et al. J Pediatr. 1999;135:465-469.
  4. Shulman S, et al. J Pediatr. 1996;129:483.
  5. Shinohara M, et al. J Pediatr. 1996;129:483-484.
  6. Shulman S, et al. J Pediatr . 2003;142:601-602.
  7. J Rheumatol. 1998;25:1215-1217.