Fatal Kawasaki Disease With Coronary Arteritis and No Coronary Aneurysms

Cardiac complications of Kawasaki disease occur in approximately 25% of patients who receive no treatment.1 The initial phase of cardiac involvement is a pancarditis with the subsequent formation of epicardial coronary aneurysms. It is well-appreciated that aneurysms may progress to stenosis, and that segmental stenoses are not uncommon in the healed forms of disease. However, diffuse narrowing of epicardial coronary arteries by a healed vasculitis has rarely been reported, and has generally been described as mild.2 We report two cases of fatal coronary vasculitis, one patient with clinical Kawasaki disease and the other without classic clinical features of mucocutaneous lymph node syndrome. In both cases death was due to diffuse fibrointimal proliferation involving all epicardial arteries resulting in >75% luminal narrowing throughout the coronary system. ### Case 1 A 4-year-old white boy had a 7-month history of multiple hospital admissions for fever and abdominal pain. The child was well until an episode of a severe cold at age 3, which was characterized by fever, conjunctivitis, pharyngitis, rash, and mild lymphadenopathy. The illness and fever subsided within 2 weeks, but soon thereafter intermittent severe abdominal pain, with episodes lasting 5 to 20 minutes, occurred. The pain resolved for several months and recurred. At this the patient was evaluated, and Coombs positive autoimmune hemolytic anemia and an erythrocyte sedimentation rate of 50 mm/hour were found. A gastrointestinal evaluation revealed an absence of gallstones, and a tentative diagnosis of ulcerative colitis was made. A diagnosis of Kawasaki disease was not considered, and a cardiac evaluation was never undertaken. The patient's physical and mental growth and development were normal. A family history was remarkable only for a maternal aunt with juvenile rheumatoid arthritis. Two weeks before death he was admitted for abdominal pain, circumoral cyanosis, and apnea. During this admission, the white blood cell count …