ARTERIAL FIBROMUSCULAR DYSPLASIA ASSOCIATED WITH SEVERE ALPHA(1)-ANTITRYPSIN DEFICIENCY

Objective: To elucidate the putative arteriopathy associated with alph a(1)-antitrypsin (alpha(1)-AT) deficiency. Design: We retrospectively studied the frequency of occurrence of fibromuscular dysplasia (FMD) i n patients with alpha(1)-AT deficiency in whom a postmortem examinatio n had been done during a 10-year period at the Mayo Clinic. Material a nd Methods: The medical records of all patients in whom an autopsy was done at the Mayo Clinic between 1983 and 1992 were reviewed to identi fy all those with a diagnosis of alpha(1)-AT deficiency or FMD. Result s: Arterial FMD was found in 2 of 6 patients with alpha(1)-AT deficien cy (33.3%; 95% confidence interval, 4.3 to 77.7%) in comparison with 2 3 of 6,690 patients without alpha(1)-AT deficiency (0.3%; 95% confiden ce interval, 0.2 to 0.5%), In both patients with alpha(1)-AT deficienc y and FMD, the arterial media was thickened and composed of irregular arrays of muscular and connective tissue fibers in a background of muc oid ground substance. Conclusion: These findings provide further evide nce for an underlying arteriopathy in patients with alpha(1)-AT defici ency and suggest that FMD may be a nonspecific disorder.