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A is for Angioedema
Shane Colliss
SCGH ED
31/10/13
• Quincke HI (1882). "Über
akutes umschriebenes
Hautödem". Monatsh
Prakt Derm 1: 129–131.

http://en.wikipedia.org/wiki/Heinrich_Quincke
:p
“I’ve got a patient over in
C18NO and he won’t stop
sticking his tongue out at
me.”
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/
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
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
Management of Angioedema
• General approach:
– ABC
• Low threshhold for intubation

– IM Adrenaline
– PO/IV steroids
– PO/IV antihistamines

• Specific management
Allergic Angioedema
• IgE mediated / Type I Hypersensitivity
• Causes:
– Food – Shellfish, Nuts, Eggs
– Drugs – Penicillin, NSAIDS, Sulphur
– Latex
– Venom - Bees

• Investigations:
– Mast cell tryptase
Mast Cell Tryptase
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
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
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?
Idiopathic Angioedema
• 38% of presentations

http://academiclifeinem.com/paucis-verbis-card-angioedema/

• Frequent, relapsing
• ?Autoimmune relationship (SLE?)

http://www.dermnetnz.org/reactions/angioedema.html
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
Hereditary Angioedema
• Not associated with urticaria
• Precipitants – Stress, trauma, hormonal
changes, exercise, alcohol
• Onset usually with puberty
• Gastrointestinal/Urological/Respiratory/Derm
atological symptoms
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
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
Who needs admission?

http://academiclifeinem.com/wp-content/uploads/2010/03/20100326Angioedema.png
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.
• 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/

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Angioedema

  • 1.
  • 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
  • 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
  • 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/

Editor's Notes

  1. 1882 - Heinrich IrenaeusQuincke (German surgeon and physician 1842-1922)– first to document and recognise angioedema. Referred to as Quinke’s oedema.
  2. ABC. Resus area.Adrenaline IM. IV access. Difficult airway trolley + ScalpelBackground – Anaphylaxis/Allergy. Drugs/Exposure/Toxins. HAE.
  3. J Emerg Med. 2013 Apr;44(4):764-72. doi: 10.1016/j.jemermed.2012.07.055. Epub 2012 Oct 28.Fresh frozen plasma for progressive and refractory angiotensin-converting enzyme inhibitor-induced angioedema.http://www.medscape.com/viewarticle/781861_1Fresh frozen plasma in the treatment of resistant angiotensin-converting enzyme inhibitor angioedema.Warrier MR, Copilevitz CA, Dykewicz MS, Slavin RG.http://www.ncbi.nlm.nih.gov/pubmed/15191027
  4. A2RB’s shouldn’t inhibit degradation of bradykinin??