Kawasaki Disease

• It is an acute self-limited febrile illness of childhood and is the most common cause of acquired heat disease in developed countries

  Kawasaki Disease
• Former names of Kawaski Disease

  Mucocutaneous lymph node syndrome

  Infantile polyarteritis nodosa
• Manifestation of Kawasaki Disease

  Vasculitis
• Risk factor for Kawasaki Disease

  Infants

  Children < 5 years old

  Asian descent

  Biologically male
• Complications of Kawasaki Disease

  Coronary artery aneurysm

  Decreased myocardial contractility - heart failure

  Myocardial infarction

  Arrhythmias

  peripheral artery occlusion
• Signs and Symptoms of Kawasaki Disease [CRASH and BURN]

  Conjunctivitis: bilateral, nonexudative

  Polymorphous Rash: desquamating

  Cervical lymphadenopathy

  Strawberry tongue: cracked red lips, oral mucositis

  Hand-foot erythema/desquamation: edema, erythema

  Fever: "burn"
• What is see in Chest x-ray in patients with Kawasaki Disease

  Cardiomegaly
• What is seen in echocardiography in patients with Kawasaki Disease

  Coronary artery aneurysm

  Pericardial effusion

  Decreased contractility
• Laboratory results in patients with Kawasaki disease

  increased CRP, ESR, platelet count (reactive thrombocytosis)
• Other diagnostics for Kawasaki disease

  Four of five CRASH symptoms

  High fever lasting 5 days
• ECG of patient with Kawasaki Disease

  Arrhythmias

  Abnormal Q waves

  Prolonged PR and QT intervals
• What are the etiologies of Kawasaki disease

  Infectious

  Genetics
• Ratio of males to females affected by Kawasaki disease

  1.5:1
• Recurrence rate of Kawasaki disease in Japan

  3%
• Relative risk in siblings

  10-fold higher
• Case fatality rate

  <0.1%
• Vasculitis is a condition that predominantly affects the medium-sized arteries. Coronary arteries may


  No change

  Transient dilatation (4-6 weeks)

  Significant pathologic consequence

  Associated findings

  Other arteries develop dilatation
• Hepatic manifestation of Kawasaki disease

  Hepatitis
• Lung manifestation of Kawasaki Disease

  Pneumonitis
• Gastrointestinal manifestation of Kawasaki Disease

  Abdominal pain

  Vomiting

  Diarrhea

  Gallbladder hydrops
• CNS manifestations of Kawasaki disease

  Aseptic meningitis
• Cardiac manifestations of Kawasaki disease

  Myocarditis

  Pericarditis

  Valvulitis
• Urinary tract manifestation of Kawasaki disease

  Pyuria
• Pancreatic manifestation of Kawasaki disease

  Pancreatitis
• Lymphatic manifestation of Kawasaki disease

  Lymphadenopathy
• Pathophysiology of Kawasaki Disease [3-phase process]

  Neutrophilic Necrotizing Arteritis

  Subacute or Chronic Vasculitis

  Luminal Myofibroblastic Proliferation
• Onset and duration of the three-phase process of KD

  Neutrophilic Necrotizing Arteritis - occur within 1st 2 weeks of illness

  Subacute or Chronic Vasculitis - occur in the first 2 weeks after fever onset, last weeks to years

  Luminal Myofibroblastic Proliferation - occurs in the first 2 weeks and persists for months to years
• This phase of KD is characterized by synchronized neutrophilic process that begins in the endothelium and moves through the coronary wall. It is a self0limited process and may progressively destroy the arterial wall into the adventitia.

  Neutrophilic necrotizing arteritis
• In neutrophilic necrotizing arteritis, this may form. There is loss of intima, media, and elastica which cannot be regenerated. The rim of the remaining adventitia can rupture or undergo sequential thrombosis that can organized, recanalized, and calcified.

  Saccular aneurysm
• What are the characteristics of 2nd stage or subacute or chronic vasculitis?

  Infiltration of lymphocytes, plasma plasma cells and eosinophils, with fewer macrophages

  Begins in the first 2 weeks after fever onset but can continue for months to years in a small subset of patients

  The vessels affected then develop LMP

  Fusiform aneurysms
• The third stage: Luminal myofibroblastic prolifertion is characterized by


  a unique smooth muscle cell divides fibroblastic process

  begins in the first 2 weeks and persists for months to years, with the potential to cause progressive arteriosclerosis.

  Large aneurysms can appear to resolve when the lumen size decreases because of layered mural thrombi or a luminal myofibroblast proliferation

  Large aneurysms can appear to resolve when the lumen size decreases because of layered mural thrombi or a luminal myofibroblast proliferation
• What are the characteristic feature of fevers associated with Kawasaki Disease

  High spiking: >38.3 degrees Celsius

  Unresponsive to antipyretics

  At least 5 days duration or may persist longer
• Kawasaki disease has at least four of the following principal features

  Changes in the extremities

  Polymorphous exanthema

  Bilateral non exudative conjunctival injections

  Changes in lips and oral cavity

  Cervical lymphadenopathy
• What are the changes in the extremities associated with KD

  erythema of the hands and feet

  indurated edema

  membranous desquamation
• What are the characteristics of the desquamation that occur in Kawasaki disease

  starting in the subungual region

  can be seen when the edema subsides

  this can occur later (2nd week of illness)
• What are the forms of the polyamorphous exanthema that is characteristic of KD

  maculopapular

  erythema multiforme

  scarlatiniform

  psoriatic-like

  urticarial

  micropustular
• What is not a characteristic feature of polyamorphous exanthema that occur in KD

  bullous or vesicular lesion
• What is the characteristic of the tongue of patients with KD

  Erythema, strawberry tongue
• Characteristic of cervical lymphadenopathy associated with KD

  usually unilateral and large (≥1.5 cm)
• Clinical Findings of KD [MPADGOU]

  Myocarditis

  Pericarditis

  Aseptic meningitis

  Diarrhea

  Gallbladder Hydrops

  Obstructive Jaundice

  Urethritis