A woman presented with a rapid onset of hypertension, angina pectoris,
peripheral vascular disease, renal involvement, and a large liver cys
t. Surgical removal of the liver cyst precipitated renal and liver fai
lure and a terminal arrhythmia, At autopsy, there was intimal fibromus
cular dysplasia involving the arteries to the heart, liver, kidneys, a
nd intestines and evidence of recent infarction of the intestines, kid
ney, and liver. This case illustrates that intimal fibromuscular dyspl
asia (FMD) can be a diffuse and rapidly progressive disease. Some trea
tments currently being evaluated for preventing restenosis following a
ngioplasty may find use in treating this uncommon disease.