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Measuring Local Anaphylaxis in Mice
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Hereditary Angioedema Attacks: Local Swelling at Multiple Sites.

Zonne L M Hofman1, Anurag Relan2, C Erik Hack3

  • 1Laboratory for Translational Immunology, University Medical Center Utrecht, Lundlaan 6, P.O. Box 85090, 3508, GA, Utrecht, The Netherlands. zonnehofman@gmail.com.

Clinical Reviews in Allergy & Immunology
|December 21, 2014
PubMed
Summary
This summary is machine-generated.

Hereditary angioedema (HAE) attacks often involve multiple body parts simultaneously, challenging the view of HAE as a localized condition. This suggests HAE symptoms may stem from a systemic process, not just local inflammation.

Keywords:
C1-inhibitor deficiencyHereditary angioedema

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Hereditary angioedema (HAE) causes recurrent swelling, potentially life-threatening.
  • Most HAE research focuses on single-site attacks, overlooking multi-location events.

Purpose of the Study:

  • To investigate the frequency, severity, and clinical course of HAE attacks involving multiple anatomical locations.
  • To better understand the systemic nature of HAE.

Main Methods:

  • Analysis of 219 HAE attacks in 119 patients from recombinant human C1-inhibitor (rhC1INH) studies.
  • Evaluation of patient-reported visual analog scale (VAS) scores and investigator symptom scores.

Main Results:

  • 28% of patients experienced multi-location HAE attacks, with up to five simultaneous sites.
  • Severe HAE attacks frequently involved multiple, anatomically unrelated body regions.

Conclusions:

  • HAE attacks commonly affect multiple body sites concurrently.
  • Findings suggest HAE is a systemic condition, contrary to the prevailing local process theory.