SEARCH RESULTS FOR: Kawasaki-Disease

Kawasaki Disease

Kawasaki Disease: Pathogenesis and clinical findings Mutations that ↑ genetic susceptibility (e.g., FCGR2A, CASP3) External trigger such as infection or environmental factor (particulate matter, seasonal airborne antigenic triggers possible) Kawasaki Disease (KD) Acute systemic, inflammatory illness affecting medium-sized arteries (especially coronary arteries), diagnosed clinically with presence of fever & 4/5 of: conjunctivitis, polymorphous rash, cervical lymphadenopathy, mucosal involvement, edema/erythema on hands/feet. KD is the most common cause of acquired heart disease in children in developed countries. Authors: Taylor Krawec, Sunni Ho, Zarrukh Baig, Nissi Wei Reviewers: Merry Faye Graff, Emily J. Doucette, Zaini Sarwar, Mandy Ang, Charissa Chen, Taj Jadavji*, Susanne Benseler*, Danielle Nelson* * MD at time of publication Acute Phase Cytokines disrupt hypothalamic thermoregulation Fever Innate immune cells around medium-sized vessels (especially coronary arteries) are inappropriately activated Neutrophils infiltrate tunica adventitia (outer vessel wall) Coronary arteritis Cytokines enter circulation & mediate systemic inflammation Cervical lymphadenopathy Immune cells infiltrate additional tissue (e.g., lymph nodes, myocardium, pericardium) Myocarditis Pericarditis Local cytokine release Inflammation of tunica media & intima (middle & inner vessel walls) Endothelial activation in small vessels ↑ vasodilation & vessel permeability ↑ Blood flow, plasma leakage & perivascular infiltration of skin & mucosa Strawberry tongue Cracked lips Edema & erythema of hands/feet Bilateral non-exudative conjunctivitis Non-vesicular rash Uveitis Patchy destruction all 3 vessel layers (necrotizing arteritis) but with vessel wall remaining intact Early coronary changes on echocardiogram Subacute Phase / Chronic Vasculitis Vessel walls lose structural integrity Coronary artery aneurysm (rarely other aneurysms) Extensive adaptive immune-mediated destruction of intima, media, elastic lamina & smooth muscle Adaptive immune cells (e.g., eosinophils, plasma cells, lymphocytes) drive ongoing inflammation Damaged vessels disrupt blood flow Thrombus formation Acute myocardial infarction** Systemic inflammation stimulates megakaryocyte proliferation in bone marrow ↑ Platelet production Thrombocytosis Inflammation damages dermis & epidermis Gradual resolution of edema from acute phase & initiation of dermal/epidermal repair Periungal desquamation (peeling of skin around nails) Luminal Myofibroblast Infiltration ↓ Baseline coronary perfusion Coronary flow cannot ↑ to match myocardial O2 demand when needed (demand ischemia) Exertional chest pain or dyspnea Myofibroblasts replace inflammatory infiltrates in vessel walls during chronic healing phase Myofibroblast proliferation thickens intima Affected arteries become narrowed (coronary arteries most common) Exercise intolerance Ischemic myocardium Coronary artery stenosis Arrhythmias is electrically unstable Legend: Pathophysiology Mechanism Sign/Symptom/Lab Finding Complications Published Oct 10, 2014; updated Dec 12 2025 on www.thecalgaryguide.com