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

Abstract

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.

Management of patients with hereditary angioedema in Germany: comparison with other countries in the Icatibant Outcome Survey

“…4 This is also supported by several international and regional consensus documents. 5 Icatibant, a bradykinin B2 receptor antagonist, was approved in the EU in 2008 for the acute treatment of HAE attacks, based upon efficacy and safety data in adults with HAE-CI-INH from two phase III randomized controlled trials. 6 Subsequently, in 2011, based on data from a phase IIIb open-label study, 7 approval was extended to self-administration.…”

Recurrent Angioedema with Abdominal and Genital Involvement in Childhood: Hereditary Angioedema Type 2 Disease due to C1 Inhibitor Functional Deficiency

“…Accordingly, both of our cases were Type 2 C1 INH-HAO. The nC1 INH-HAO group, which is more common in adolescents and adults, has not been reported much in childhood in the literature (9,10).…”

“…Another treatment approach in cases with HAO is short-term prophylaxis to prevent acute attacks during interventions such as tooth extraction or surgical operation known to trigger attacks or immediately after trauma. FFP, weak androgens and C1 INH replacement can also be used for short-term prophylaxis (10). In our country, drugs such as weak androgens and anti-fibrinolytics can be used for short or long term prophylaxis, but C1-INH products from plasma are not covered by SGK.…”

Unnecessary abdominal interventions in patients with hereditary angioedema

“…Die in der Literatur geschätzte Prävalenz der Krankheit reicht von 1 : 10.000 bis 1 : 150.000 [8][9][10] . HAE betrifft alle ethnischen Gruppen [ 11 ] . In einer Studie der Icatibant Outcome Survey Group in sechs europäischen Ländern war die Verteilung hinsichtlich Geschlecht und HAE-Typ in allen Ländern ähnlich, mit einem Überwiegen der Frauen, wie auch Maurer et al feststellten [ 12,13 ] .…”