Arterial Fibromuscular Dysplasia Associated With Severe α1-Antitrypsin Deficiency

WOUTER I. SCHIEVINK, JOHANNES BJÖURNSSON, JOSEPH E. PARISI, UDAYA B.S. PRAKASH

Research output: Contribution to journalArticle

47 Scopus citations

Abstract

To elucidate the putative arteriopathy associated with α1-antitrypsin (α1-AT) deficiency. 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. 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. 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 (03%; 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. 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
DOIs
StatePublished - Jan 1 1994

Keywords

  • CI
  • FMD
  • confidence interval
  • fibromuscular dysplasia
  • α -AT
  • α -antitrypsin

ASJC Scopus subject areas

  • Medicine(all)

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    SCHIEVINK, WOUTER. I., BJÖURNSSON, JOHANNES., PARISI, JOSEPH. E., & PRAKASH, UDAYA. B. S. (1994). Arterial Fibromuscular Dysplasia Associated With Severe α1-Antitrypsin Deficiency. Mayo Clinic proceedings, 69(11), 1040-1043. https://doi.org/10.1016/S0025-6196(12)61369-X