What is kawasaki disease? pathogenesis, morphology and clinical features

 Kawasaki Disease

Kawasaki disease is an acute, febrile, usually self-limited illness of infancy and childhood associated with an arteritis of mainly large- to medium-sized vessels.

Approximately 80% of patients are younger than 4 years of age. Its clinical significance stems from the involvement of coronary arteries. Coronary arteritis can result in aneurysms that rupture or thrombose, causing myocardial infarction. Originally described in Japan, the disease is now recognized in the United States and elsewhere.

What is kawasaki disease?


In genetically susceptible individuals, a variety of infectious agents (mostly viral) have been posited to trigger the disease. The vasculitis may result from a delayed-type hypersensitivity response directed against cross-reactive or newly uncovered vascular antigen(s). Subsequent cytokine production and polyclonal B cell activation result in auto-antibodies to ECs and SMCs that precipitate the vasculitis.

Morphology of Kawasaki disease

  • Affects medium-sized arteries, especially coronary arteries.

  • Acute phase: Necrotizing vasculitis with neutrophil infiltration.

  • Subacute phase: Mononuclear cell infiltration and intimal thickening.

  • Chronic phase: Luminal myofibroblastic proliferation → stenosis.

  • Coronary artery aneurysms may form due to wall weakening.

  • Myocarditis, pericarditis, and endocarditis can occur.

  • Skin and mucous changes: Peeling skin, red hands/feet, conjunctivitis, "strawberry tongue."

Pathogenesis of Kawasaki Disease (KD):

  • Unknown trigger (possibly infectious) in genetically predisposed children.

  • Activates immune system → cytokine release.

  • Causes widespread inflammation of medium-sized arteries (vasculitis).

  • Endothelial damage → aneurysm formation, thrombosis, or stenosis.

  • Autoimmune response may contribute to ongoing vessel injury.

Clinical Features

Kawasaki disease typically manifests with conjunctival and oral erythema and blistering, edema of the hands and feet, erythema of the palms and soles, a desquamative rash, and cervical lymph node enlargement (hence its other name, mucocutaneous lymph node syndrome). Approximately 20% of untreated patients develop cardiovascular sequelae, ranging from asymptomatic coronary arteritis to coronary artery ectasia to large coronary artery aneurysms (7 to 8 mm in diameter); the latter may be associated with rupture, thrombosis, myocardial infarction, and/or sudden death. Treatment consists of intravenous immunoglobulin infusions (which suppress inflammation through unclear mechanisms) and aspirin, which, when given together, markedly decrease the incidence of symptomatic coronary artery disease.

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