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- Kawasaki disease
- mucocutaneous lymph node syndrome
- coronary artery disease
- coronary aneurysm
- coronary angiography
- coronary stenting
- gene association
A 37-year-old man presented with progressive angina. He had never smoked, and did not have diabetes mellitus, kidney disease or familial hypercholesterolaemia. An exercise test showed significant lateral and inferior ST-segment depression with chest pain. His past medical history was notable for a severe sepsis at the age of 13 years, with prolonged fever, jaundice and desquamation of the palms. No firm diagnosis could be made.
A right coronary artery angiogram revealed ‘arteries in the artery’ in the proximal part, a sign of spontaneous recanalisation of a previously totally occluded artery (figure 1 and online supplementary video 1). The left coronary artery angiogram showed severe stenosis of the distal left main coronary artery, left anterior descending artery and left circumflex artery, with calcified saccular aneurysms. Distal segments were angiographically normal. Calcifications of the coronary aneurysms were present and visible by plain radiography (figure 2 and online supplementary video 2).
These coronary lesions are typical coronary sequels of Kawasaki disease. Also termed mucocutaneous lymph node syndrome, Kawasaki disease is a vasculitis of unknown aetiology, occurring mostly in infancy and early childhood. Approximately 15% to 25% of the patients, who are not treated early, develop coronary artery aneurysms, mostly located on the proximal coronary arteries, with a predilection for branching points. The acute phase of the illness is self-limited and the diagnosis can be missed, as it probably was in this patient. There is no specific diagnostic test. In retrospect, the severe sepsis at the age of 13 years was most likely the acute episode of Kawasaki disease. The patient underwent a coronary artery bypass graft and recovered uneventfully.
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; internally peer reviewed.
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