This document provides an overview of angioedema, beginning with its definition as a self-limited, asymmetric swelling caused by increased vascular permeability. It describes different types of angioedema including allergic, drug-induced, idiopathic, and hereditary forms. Management is discussed, focusing on the ABCs - securing the airway, using adrenaline for laryngeal edema, and administering steroids, antihistamines, or specific treatments depending on the underlying cause. Factors that warrant admission are noted.

2. A is for Angioedema
Shane Colliss
SCGH ED
31/10/13

3. • Quincke HI (1882). "Über
akutes umschriebenes
Hautödem". Monatsh
Prakt Derm 1: 129–131.
http://en.wikipedia.org/wiki/Heinrich_Quincke

4. :p
“I’ve got a patient over in
C18NO and he won’t stop
sticking his tongue out at
me.”

5. Angioedema
• Self limited, asymmetric, localised, non-pitting
swelling
• Results from increased vascular permeability
• Affects lips, eyes, tongue, respiratory and GI
mucosa
– Less connective tissue = Increased capacity to
swell
• Laryngeal oedema = 25-40% mortality
http://academiclifeinem.com/paucis-verbis-card-angioedema/
http://lifeinthefastlane.com/traumatic-angioedema/

6. Shane, did you say Urticaria or
Angioedema?
• Urticaria
– Epidermal and dermal oedema
– Redness and raised areas on skin
only
– Itchy. Rarely painful.
• Angioedema
http://www.virtualmedicalcentre.com/diseases/hives-urticaria/762
– Subcutaneous and mucosa
oedema
– Swelling below skin and mucosa
– Often painful. Sometimes itchy.
http://www.dermnetnz.org/reactions/angioedema.html
http://en.wikipedia.org/wiki/File:Angioedema2013.JPG

7. Types of Angioedema
•
•
•
•
•
Allergic / IgE mediated angioedema
Non-allergic drug induced angioedema
Idiopathic Angioedema
Hereditary Angioedema
Acquired C1 inhibitor deficiency
http://www.dermnetnz.org/reactions/angioedema.html

8. Management of Angioedema
• General approach:
– ABC
• Low threshhold for intubation
– IM Adrenaline
– PO/IV steroids
– PO/IV antihistamines
• Specific management

9. Allergic Angioedema
• IgE mediated / Type I Hypersensitivity
• Causes:
– Food – Shellfish, Nuts, Eggs
– Drugs – Penicillin, NSAIDS, Sulphur
– Latex
– Venom - Bees
• Investigations:
– Mast cell tryptase

10. Mast Cell Tryptase

11. Non-allergic Drug Induced
Angioedema
• Commonly ACE inhibitors
– ACE inhibitors inhibit ACE
• ACE normally degrades bradykinin
• Bradykinin causes vasodilation and increases
vascular permeability
• Inhibition results in rapid accumulation of
fluid in the interstitial space

12. ACEi Induced Angioedema
• Approximately 2% of patients are affected
• Higher incidence in first month of use
•
•
•
•
•
•
Antihistamines won’t work
Adrenaline will* work for laryngeal oedema
FFP – Try two units
Icatibant – Bradykinin B2 receptor antagonist
Ecallantide – Kallikrein inhibitor
Recombinant C1-Inhibitor
http://www.ncbi.nlm.nih.gov/pubmed/15191027

13. ACEi induced angioedema
• “…cessation of the ACEI is necessary to reduce
the risk of recurrent episodes.”
– http://www.racgp.org.au/download/documents/A
FP/2011/December/201112andrew.pdf
• A2RB safety?

14. Idiopathic Angioedema
• 38% of presentations
http://academiclifeinem.com/paucis-verbis-card-angioedema/
• Frequent, relapsing
• ?Autoimmune relationship (SLE?)
http://www.dermnetnz.org/reactions/angioedema.html

15. Hereditary Angioedema
•
•
•
•
Autosomal dominant
Type 1 = Reduced level and function of C1-INH
Type 2 = Normal level, poor function of C1-INH
Type 3 = Factor XII gene coding mutation
• Reduced C1 esterase inhibitor activity 
excessive kallikrein  excessive bradykinin
production

16. Hereditary Angioedema
• Not associated with urticaria
• Precipitants – Stress, trauma, hormonal
changes, exercise, alcohol
• Onset usually with puberty
• Gastrointestinal/Urological/Respiratory/Derm
atological symptoms

17. Hereditary Angioedema
• World Allergy Organisation guidelines:
– Test for C4, C1-INH protein, C1-INH function
– Treat with:
• Icatibant – Bradykinin B2 receptor antagonist
• Ecallantide – Kallikrein inhibitor
• Recombinant C1-Inhibitor
– Treat early if airway involvement +/- ETT/trache
– HAE 1/2 patients should carry “on demand” treatment
for two attacks
– Prophylaxis:
• Androgen (danazol, stanazolol) increase circulating C1-INH
http://emedicine.medscape.com/article/135208-overview

18. Acquired C1 inhibitor deficiency
• Associated with:
– B Cell Lymphoma
– C1 Inhibitor antibodies
http://www.dermnetnz.org/reactions/angioedema.html
• Diagnosed at advanced age
• No family history
• Treatment as for HAE

19. Who needs admission?
http://academiclifeinem.com/wp-content/uploads/2010/03/20100326Angioedema.png

20. ABC’s of Angioedema
•
•
•
•
•
•
A is for Airway and Adrenaline
B is for Bradykinin
C is for C1 Esterase Inhibitor
D is for Drug induced – ACEi, gliptins
E is for EpiPen prescription
F is for FFP x2
Thanks for listening. I hope you’ve had a swell time.

21. • Title image: http://thechive.files.wordpress.com/2013/10/pumpkincarving-3.jpg?w=500&h=375
• http://www.dermnetnz.org/reactions/angioedema.html
• http://academiclifeinem.com/paucis-verbis-card-angioedema/
• http://lifeinthefastlane.com/education/ccc/angioedema/