Kawasaki disease is an acute self-limited vasculitis of childhood affecting medium-sized vessels, particularly coronary arteries. The feared complication is coronary artery aneurysm. Which pathological sequence explains aneurysm formation in Kawasaki disease?
- A Neutrophil-mediated destruction of all three arterial wall layers followed by fibromyxoid tissue replacement weakening the wall ✓
- B Immune complex deposition in vessel wall activating complement causing membrane attack complex lysis
- C CD8+ T-cell cytotoxic killing of arterial smooth muscle cells
- D Septic emboli seeding the vasa vasorum causing necrotising arteritis
Explanation
In Kawasaki disease, an initial intense neutrophilic infiltration of the vessel wall progresses to a subacute/chronic phase with mononuclear cells (CD8+ T cells, macrophages) destroying all three arterial layers (intima, media, adventitia). Matrix metalloproteinase secretion by inflammatory cells degrades elastin and collagen in the tunica media, losing the structural integrity of the vessel wall. The destroyed muscular wall is replaced by fibromyxoid tissue unable to withstand arterial pressure, leading to aneurysm formation — typically at the proximal coronary artery segments. IVIG reduces this risk by suppressing the inflammatory cascade.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
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Written and medically reviewed by the StethoPrep medical team.