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Hereditary angioedema and aortitis.

U Hoffmann1, J Buss, V Voigtlaender

  • 1Innere Medizin, Universität Heidelberg, Klinikum Mannheim.

Klinische Wochenschrift
|September 15, 1987
PubMed
Summary
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Hereditary angioedema (HAE) may be linked to aortic inflammation and stroke. An autopsy revealed cicatricial aortitis and myocardial infarctions in a patient with HAE, suggesting a potential association.

Area of Science:

  • Cardiovascular Pathology
  • Immunology
  • Neurology

Background:

  • Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent, unpredictable swelling attacks.
  • The potential systemic complications of HAE beyond angioedema are not fully understood.
  • Aortic pathology has not been previously associated with HAE.

Observation:

  • A 28-year-old male with a known diagnosis of hereditary angioedema experienced a fatal extensive stroke.
  • Autopsy examination revealed cicatricial aortitis with significant narrowing of the coronary ostia.
  • Evidence of myocardial infarctions and a left ventricular mural thrombus were also identified.

Findings:

  • The autopsy findings demonstrated chronic aortic inflammation (cicatricial aortitis) without signs of acute inflammation or systemic vasculitis.

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  • Coronary ostial stenosis secondary to aortitis likely contributed to myocardial ischemia and infarction.
  • The presence of a left ventricular mural thrombus is a recognized complication of myocardial infarction.

    • Implications:

    • This case suggests a potential, previously unrecognized association between hereditary angioedema and the development of cicatricial aortitis.
    • The aortic pathology may represent a systemic manifestation of HAE, contributing to cardiovascular complications such as stroke and myocardial infarction.
    • Further research is warranted to investigate the underlying mechanisms linking HAE to aortitis and to assess the cardiovascular risk in HAE patients.