Angioedema

A is for Angioedema

Shane CollissSCGH ED31/10/13

• 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– Subcutaneous and mucosa

oedema– Swelling below skin and mucosa– Often painful. Sometimes itchy.

http://en.wikipedia.org/wiki/File:Angioedema2013.JPG

http://www.dermnetnz.org/reactions/angioedema.html

http://www.virtualmedicalcentre.com/diseases/hives-urticaria/762

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/AFP/2011/December/201112andrew.pdf

• A2RB safety?

Idiopathic Angioedema

• 38% of presentations

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

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

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/Dermatological 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-INHhttp://emedicine.medscape.com/article/135208-overview

Acquired C1 inhibitor deficiency

• Associated with:– B Cell Lymphoma– C1 Inhibitor antibodies

• Diagnosed at advanced age• No family history

• Treatment as for HAE

http://www.dermnetnz.org/reactions/angioedema.html

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/pumpkin-carving-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/