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angioedema
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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/
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