The present study was done to investigate the relation between the prognosis and hypertension, especially hypertension due to renal arterial stenosis, atypical coarctation of the aorta and aortic regurgitation in patients with aortitis syndrome. Sixty-five patients including 25 with normotension and 40 with hypertension who were hospitalized during the last 20 years were included in this study. Out of the 40 patients with hypertension, a cause of hypertension was found in 37 patients: renovascular hypertension alone in 13, renovascular hypertension with atypical coarctation in 11, renovascular hypertension and aortic regurgitation in 2, atypical coarctation alone in 5, aortic regurgitation alone in 5 and all of the three in the remaining one. Thirty-two hypertensive patients out of 40 were treated with antihypertensive drugs and the remaining 8 underwent surgical operation (renal vascular repair in 6 and nephrectomy in 2 for relief of hypertension. In the present data, 13 out of 40 patients with hypertension died despite of the treatment, while only one out of 25 patients with normotension died. According to the mortality in each type of cardiovascular lesion, 6 patients died out of 13 with renovascular hypertension alone, 5 patients died out of 11 with renovascular hypertension associated with atypical coarctation and 2 patients with renovascular hypertension associated with aortic regurgitation died. In contrast, there was no death in hypertensive patients with aortic regurgitation alone or atypical coarctation of the aorta alone. These results indicate that hypertension, especially renovascular hypertension is one of the major determinant of the prognosis in the patient with aortitis syndrome.