Fatal Kawasaki Disease (KD) due to Early Fibrous Obliterative Coronary Artery Disease

Abstract

The purpose of the study is to describe 2 fatal cases of KD due to nonthrombotic obstructive coronary artery disease. Patient 1. 4yr Caucasian male, URTI, low grade fever, eventually had 4 of 5 KD criteria. Given IVIG x 2 about day 15 with no response: persistent fevers and hemolytic anaemia 1 month, small joint arthritis & arthralgias 2nd month, abdominal pains 3rd month. Initial coronary artery (CA) dilatation progressed with the development of unstable angina and low cardiac output 4 months after presentation. Died after anaesthetic induction for cardiac catheterization. Post mortem histology showed thick walled obstructed triple vessel CA disease. Histology showed marked fibrocellular intimal proliferation. There was recent myocardial and duodenal infarction. Patient 2. 6 month Caucasian male developed fever and 4 criteria for KD but no peripheral oedema or redness. Given IVIG day 8 and day 20 due to irritability without fever. Echo day 8 and day 50 showed mild CA dilatation. He represented day 95 with congestive heart failure, cardiogenic shock, poor LV function and died. Post mortem showed minor CA aneurysmal changes only. There was marked luminal obstruction caused by replacement fibrosis of the intimal and media. There was patchy chronic inflammatory infiltration.In summary, these 2 patients showed atypical KD histology with early intimal fibrosis and luminal obstruction. Conclusions:1. KD may be fatal due to early (within 3-4 months from onset) fibrotic obliterative CA disease rather than due to giant CA aneurysms and complications.2. New treatment strategies for KD are required for late presenters or non-responders to IVIG when there is evidence of continued disease process.