Arterial fibromuscular dysplasia associated with severe α1-antitrypsin deficiency

W. I. Schievink, J. Bjornsson, Joseph E Parisi, U. B S Prakash

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Abstract

Objective: To elucidate the putative arteriopathy associated with α1- antitrypsin (α1-AT) deficiency. Design: We retrospectively studied the frequency of occurrence of fibromuscular dysplasia (FMD) in patients with α1-AT deficiency in whom a postmortem examination had been done during a 10-year period at the Mayo Clinic. Material and Methods: The medical records of all patients in whom an autopsy was done at the Mayo Clinic between 1983 and 1992 were reviewed to identify all those with a diagnosis of α1-AT deficiency or FMD. Results: Arterial FMD was found in 2 of 6 patients with α1-AT deficiency (33.3%; 95% confidence interval, 4.3 to 77.7%) in comparison with 23 of 6,690 patients without α1-AT deficiency (0.3%; 95% confidence interval, 0.2 to 0.5%). In both patients with α1-AT deficiency and FMD, the arterial media was thickened and composed of irregular arrays of muscular and connective tissue fibers in a background of mucoid ground substance. Conclusion: These findings provide further evidence for an underlying arteriopathy in patients with α1-AT deficiency and suggest that FMD may be a nonspecific disorder.

Original languageEnglish (US)
Pages (from-to)1040-1043
Number of pages4
JournalMayo Clinic Proceedings
Volume69
Issue number11
StatePublished - 1994

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ASJC Scopus subject areas

  • Medicine(all)

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Schievink, W. I., Bjornsson, J., Parisi, J. E., & Prakash, U. B. S. (1994). Arterial fibromuscular dysplasia associated with severe α1-antitrypsin deficiency. Mayo Clinic Proceedings, 69(11), 1040-1043.