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Key Features

Essentials of Diagnosis

  • Fever, conjunctivitis, oral mucosal changes, rash, cervical lymphadenopathy, peripheral extremity changes

  • Elevated erythrocyte sedimentation rate and C-reactive protein levels

  • Affects medium-sized arteries and multiple organs, causing

    • Interstitial pneumonitis

    • Abdominal pain

    • Diarrhea

    • Gallbladder hydrops

    • Pancreatitis

    • Pyuria

General Considerations

  • Also known as the "mucocutaneous lymph node syndrome"

  • An acute, self-limiting, mucocutaneous vasculitis characterized by

    • Infiltration of vessel walls with mononuclear cells

    • IgA secreting plasma cells that can destroy the tunica media and cause aneurysm formation

  • Seen mainly in children between the ages of 3 months and 5 years but can occur occasionally in adults

  • Occurs more significantly in Asians or native Pacific Islanders

  • Risk factors

    • Advanced maternal age

    • Mother of foreign birth

    • Maternal group B streptococcus colonization

    • Early infancy hospitalization for a bacterial illness

    • Genetic factors

  • Cause remains unknown

Clinical Findings

  • A clinical diagnosis of classic or "complete" Kawasaki disease requires the presence of at least 5 days of fever, usually high-grade (over 39–40°C) and four of the following five criteria:

    • Bilateral nonexudative conjunctivitis (begins shortly after the onset of fever)

    • Oral changes

      • Erythema and cracking of lips

      • Strawberry tongue

      • Erythema of oral and pharyngeal mucosa

    • Peripheral extremity changes

      • Erythema and edema of the hands and feet in the acute phase

      • Periungual desquamation within 2–3 weeks after the onset of fever

    • Polymorphous rash

    • Cervical lymphadenopathy

      • > 1.5 cm diameter

      • Usually unilateral

      • Least common of the clinical features

  • Revised case definition allows the diagnosis on day 4 in the presence of more than 4 clinical criteria, particularly when redness and swelling of the hands and feet are present

  • A diagnosis of atypical or "incomplete" Kawasaki disease can be diagnosed in patients with unexplained fever and fewer than 4 criteria if accompanied by

    • Compatible laboratory findings or

    • Aneurysms detected by echocardiography or angiography

Differential Diagnosis

  • Measles in unimmunized children

  • Other viral infections, such as adenovirus, scarlet fever and toxic shock syndrome

  • Rickettsial infections or leptospirosis

  • Drug hypersensitivity reactions

Diagnosis

  • In the acute phase, following findings are typical:

    • Leukocytosis with neutrophilic predominance

    • Anemia

    • Elevated erythrocyte sedimentation rate (ESR)

    • C-reactive protein (CRP)

  • High platelet counts are characteristic but occur in the second week

  • N-terminal moiety of B-type natriuretic peptide (NT-proBNP)

    • May be elevated in some patients

    • Likely indicative of myocardial involvement

  • CSF pleocytosis with a mononuclear cell predominance, normal glucose levels, and protein levels is seen in one-third of children who undergo lumbar puncture

Treatment

  • Intravenous immunoglobulin (IVIG)

    • Single dose should be given in the first 10 days of the illness

    • If diagnosis is made later than the tenth day, IVIG may still beneficial if patient has elevated inflammatory markers (ESR or CRP) with persistent fever or coronary artery aneurysms

    • When IVIG treatment is not given, coronary artery aneurysms occur in 1 in 5 children

    • It ...

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