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Angioedema ED Guideline

Angioedema:
including Hereditary Angioedema (HAE)& ACEinhibitor induced Angioedema
(ACEi Angioedema)
Written 30/11/2015 Dr Rob Greig. Jersey ED

Background:
HAE is a rare autosomal dominant disease, of which there are 3 forms: Type 1, 85% of HAE,
characterised by low levels of C1 Esterase Inhibitor (C1inh) and levels of dysfunctioning C1inh.
Type 2 HAE has dysfunctioning C1inh. Type 3 HAE is characterised by recurrent, self limiting
episodes of Angioedema but with normal levels of functioning C1inh.
The mediators responsible for swelling in HAE with normal C1inh (Type 3 disease) has not been
definitely identified. Excess generation of bradykinin has convincingly been shown to be the major
cause of the angioedema in Types 1&2. Activation of the kinin system is believed to be the trigger
in Type 3 disease but this hasn't been proven.
ACE inhibitor Angioedema occurs in up to 1% of patients taking ACE inhibitors (ACEi). The
symptoms can occur anytime after the first dose to many years of taking ACEi. The
pathophysiology is believed to be the reduced degradation of bradykinin with up to a tenfold
increase of this potent vasodilator that also increases vascular permeability.
ACEi Angioedema can occur weeks to months after stopping ACEi.
C1inh is an acute-phase agent and is both an inhibitor of the complement and coagulation
cascade.

Clinically:
Well demarcated Angioedema without Urticaria which affects the skin & mucosa of the upper
respiratory and GI tract
Swelling can resolve spontaneously between 12hrs - 4days without treatment
More likely in females
Does not respond to Adrenaline or Steroids
Often no trigger
PMH of swelling attacks
Can cause life threatening massive Laryngeal or Pharyngeal oedema.

Dr Rob Greig, Nov 2015

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Angioedema ED Guideline

Management:
ABC assessment initially
Unlike Anaphylaxis the blood pressure doesn't drop
Patients with HAE may carry with them either IV C1esterase Inhibitor or S/C Icatibant (Firazyr trade name)
Call for senior help: if out of hours call anaesthetic support early
Start nebuliser adrenaline and if in doubt give IM Adrenaline 0.5mg - HAE/ACEi Angioedema
doesn't respond but it may be Anaphylaxis and topical adrenaline in the upper airway will cause
some vasoconstriction (remember the pathophysiology involves vasodilation & increased
vascular permeability)
Give steroids: as it may be anaphylaxis and doesn't harm if it's HAE/ACEi
Specialist drugs:
Icatibant 30mg s/c (pre filled syringe,vol 3ml, abdominal wall) can be repeated after 6hrs
and a third dose can be administered after another 6hrs or
C1 Esterase Inhibitor IV (1000 i/u for weight up to 50kg, 1500 i/u up to 75kg, 2000 i/u
up to 100kg and 2500 i/u if weight greater than 100kg)- slow IV over 10mins (do not
shake vials as it denatures the protein) - works in 30mins. Can be repeated after 2 hrs
FFP. Considered 2nd line if above two agents are not immediately available. Dose is
2units initially IV over 30mins. Repeat every 2-4hrs until there is clinical improvement.
Alternative dosing: 10-15ml/kg if fluid overload an issue.
Other treatments: abdominal cramps- IM Buscopan 20mg, N&V IV Metoclopramide 10mg

Any suggestion of laryngeal oedema e.g. Stridor or Respiratory distress warrants prompt senior
anaesthetic review and consideration for pre-emptive intubation. As a precaution the ENT consultant
on call should also be informed incase a surgical airway is required.

The patient should be admitted to HDU if not intubated and ITU if intubated.

Source:
1. UpToDate updated August 2015: Marco Cicardi MD & Bruce Zuraw MD.
2. Emergency Medicine News: James R. Roberts MD. March 2014

Dr Rob Greig, Nov 2015