Review of Select Practice Parameters, Evidence-Based Treatment Algorithms, and International Guidelines for Hereditary Angioedema

Clin Rev Allergy Immunol. 2016 Oct;51(2):193-206. doi: 10.1007/s12016-016-8546-7.


Hereditary angioedema (HAE) is a rare bradykinin-mediated disease that is characterized by recurrent attacks of subcutaneous or submucosal edema, which can be life threatening. HAE affects all ethnic groups equally and does not differentiate between age, sex, or race. However, the availability of therapies varies amongst countries resulting in a lack of uniformity of care. Not only is there a disparity of medication availability, but since HAE is a rare disease, it is frequently overlooked and the diagnosis is missed. Even with diagnosis, treatment and management is often less than optimal. For these reasons, it is essential to have practice parameters and guidelines. In this chapter, we focus on recent guidelines. These guidelines deal with recognition, diagnosis, medical care, patient management, and assessment, all which are essential to provide optimal care to people with a rare and orphan disease. The intent of the guidelines, and thus this chapter, is to reduce morbidity and mortality, and restore a normal quality of life for the patient with HAE. We will review the guidelines from various regions of the world as well as international group recommendations. In addition, specific patient populations such as the pregnant, elderly, and juvenile require modified treatment regimens, and for this reason, we have included these data as well. The intent of this chapter is to aid the practitioner in holistic care of the patient with HAE in order to ultimately provide the best standard of care possible.

Keywords: Androgens; Angioedema; C1 esterase inhibitor; Children; Guidelines; HAE prophylaxis; Hereditary angioedema (HAE); Practice parameter; Pregnancy; Type 2 HAE; Type I HAE.

Publication types

  • Review

MeSH terms

  • Algorithms
  • Angioedemas, Hereditary / diagnosis*
  • Angioedemas, Hereditary / prevention & control
  • Angioedemas, Hereditary / therapy*
  • Diagnostic Techniques and Procedures
  • Disease Management
  • Humans
  • Practice Guidelines as Topic
  • Premedication
  • Risk Factors