Hereditary Angioedema

  • Saumya MaruEmail author
  • Timothy Craig
Living reference work entry


Hereditary angioedema (HAE) is a rare disease affecting approximately 1 in 50,000 people and presents with recurrent cutaneous and mucosal membrane swelling. The result is recurrent angioedema, intermittent abdominal obstruction and pain, and airway swelling. Though death is rare in patients diagnosed and on therapy, upper airway swelling can be fatal. Disability and absenteeism secondary to the frequent attacks lasting up to 3 days can limit quality of life and education and occupational stability. Multiple therapies have been approved in the last decade and have made drastic improvements in this orphan disease. This chapter will discuss epidemiology, signs and symptoms, differential diagnosis, diagnosis, treatment, and management of the HAE patient.


Hereditary angioedema (HAE) Angioedema Bradykinin C1 inhibitor (C1-INH) C1-esterase inhibitor 


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Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Penn State College of MedicineHersheyUSA
  2. 2.Department of Medicine and PediatricsPenn State College of MedicineHersheyUSA

Section editors and affiliations

  • Tim Craig
    • 1
  • Massoud Mahmoudi
    • 2
  1. 1.Penn State UniversityHersheyUSA
  2. 2.Department of MedicineUniversity of CaliforniaSan FranciscoUSA

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