Diagnosing HAE

An Expert Approach to Diagnosing HAE: Clinical Criteria and Laboratory Evaluation

Physician/researcher and UC San Diego Professor of Medicine Dr. Bruce L. Zuraw has published a formidable body of Hereditary Angioedema (HAE) work that spans a wide range of topics, including studies of molecular genetics, clinical syndrome, pathophysiology, and emergent therapies. Here we provide highlights and excerpts from Professor Zuraw’s work that elucidate his diagnostic approach.

  • Clinical Characteristics Suggesting HAE as a Potential Diagnosis (1)

    • Angioedema does not respond to treatment with epinephrine, antihistamines, or corticosteroids.
    • Attacks are prolonged, typically increasing over the first 24 hours then slowly subsiding over the next 48-72 hours before full resolution is achieved.
    • Attacks may be preceded or accompanied by a non-pruritic, flat, erythematous mottling or erythema marginatum.
    • Age of onset is variable ranging from early childhood to adult; frequency worsening around puberty.
    • The patient may or may not report a family history because up to 25 percent of new HAE cases result from de novo mutations.

    (1) Zuraw BL. Hereditary angioedema. New England Journal of Medicine. 2008;359(10):1027

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