A CLINICOPATHOLOGICAL STUDY OF FAMILIAL ABDOMINAL AORTIC-ANEURYSMS

The purpose of this report is to document what we have observed in pat ients with familial abdominal aortic aneurysms (FAAAs) between 1987 an d 1993. Patients with FAAAs were reviewed and compared with those with out familial clustering with regard to age, sex, past history, laborat ory data, smoking habits, and type of implanted graft. We identified 7 families among which a total of 15 members had AAAs. The incidence of familial clustering reached 5.4%. The mean age of the FAAA group was significantly younger than that of the non-FAAA group (mean age: 65.8/-10.3 versus 71.0+/-7.3 years). In the FAAA group, furthermore, patie nts in the second generation were significantly younger than those in the first generation (mean age: 55.3+/-10.5 versus 69.6+/-7.4 years). FAAA was significantly more often complicated by ischemic cardiac dise ases. There were no significant differences in other risk factors. Int erestingly, however, we observed a morphological similarity in the sha pe of the aneurysms within each family. Histological examinations show ed moderate or severe lymphocytic infiltration into the aortic adventi tia in 6 out of 9 cases. FAAA is clinically important, because it can represent a high-risk group that may benefit from a screening program for early detection and elective management of AAA. Studies of FAAAs w ill be useful for elucidating the pathogenesis of AAAs.