Lie, J. T.

Abstract The authors describe three cases (two blacks and one Latin American) of sudden death caused by late complications of Kawasaki disease (mucocutaneous lymph node syndrome). At autopsy each heart contained multiple coronary artery aneurysms with luminal stenosis caused by intimal hyperplasia and thrombi. Although virtually all fatal cases of Kawasaki disease occur within six months of the onset of symptoms, there have been other reported deaths up to 14 years after the acute illness. The coronary artery aneurysms of Kawasaki disease may persist and cause death years after the acute illness.

The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) is a variant but not a benign form of scleroderma. Pulmonary hypertension without pulmonary fibrosis appears to be more prevalent in the CREST than in scleroderma patients. Moreover, pulmonary hypertension in CREST may be rapidly progressive and a cause of sudden death from severe cor pulmonale.

The hazard of rupture of asymptomatic splenic artery aneurysms in preg nancy is well known. Arterial dysplasia is an important etiologic basis of splenic artery aneurysms in women of child-bearing age, as exemplified by the two cases described. In one patient, multiple splenic artery aneurysms oc curred in association with hypoplasia of the aorta and visceral arteries. In another patient, a false aneurysm of the splenic artery occurred with coexisting fibromuscular dysplasia of the renal arteries.

Thromboangiitis obliterans (Buerger's disease) is an occlusive vascular disease that occurs almost exclusively in young male tobacco users. A unique case is documented here in which Buerger's disease affecting the upper limbs was diagnosed in a sixty-two-year-old man who was a cigarette smoker but had stopped smoking fifteen years earlier.