Anatomy of the posterior pharynx.

Angioedema is paroxysmal, nondemarcated swelling of dermal or submucosal layers of skin or mucosa

Swelling is asymmetric, nonpitting, and nonpruritic, however can be associated with allergic features depending on cause

Isolated uvular angioedema, or Quincke's disease, is a relatively rare presentation of angioedema of the upper airway

Uvular Angioedema (Quincke's Disease)

Bradykinin-mediated

Hereditary angioedema: Congenital or acquired loss of C1 esterase inhibitor

Due to C1 esterase inhibitor deficiency

Leads to unregulated activity of vasoactive mediators (bradykinin) associated with complement pathway

Autosomal dominant

ACE-I-induced angioedema: ACE-I adverse reaction from excessive bradykinin

Idiopathic

Angioedema of face.

Angioedema

Angioedema of tongue

Suspect in patients with history of recurrent peripheral angioedema and abdominal pain

75% experience onset of symptoms before age 15yr

C4 level screens for HAE (suspect if low)

Decreased levels of C1 and C4 esterase inhibitors confirms diagnosis

Incidence is highest within the first month; however, may occur at anytime

40% present months to years after initial dose

Incidence is 0.1-2.2% (more common in blacks)

Physiology more closely related to bradykinin-mediated pathway than IgE-mediated pathway, therefore current treatments may be insufficient

Consider definitive airway if voice change, hoarseness, stridor, dyspnea

Ecallantide

10mg SQ x 3 in different anatomical locations (30mg in total)

Icatibant

30mg SQ

Consider Tranexamic acid (1 g IV, over 10 min) for bradykinin-mediated angioedema

Icatibant

30mg SQ

Significantly decreases time to complete resolution (8 hrs vs 27.1 hrs)

Note: control group did not receive FFP

Consider Ecallantide

10mg SQ x 3 in different anatomical locations (30mg in total)

A 2015 trial showed a non-statistically significant trend towards increased rate of ED discharge

No benefit seen in mild-moderate cases in multi-center, double blind study

Consider discharge after 4-6 hrs observation if there is no airway edema and patient improves or swelling remains isolated to face and lips only

24 hrs obs if epinephrine given

Ishoo Staging may predict severity of disease to help stratify disposition. It is based on retrospective study

Apneic oxygenation

Induction

Intubation

Non-traditional intubation

Surgical airways

Post-intubation

https://www.merckmanuals.com/professional/immunology-allergic-disorders/allergic,-autoimmune,-and-other-hypersensitivity-disorders/angioedema?query=angioedema

Winters ME, et al. Emergency department management of patients with ACE-inhibitor angioedema. JEM. 2013; 45(5):775–780.

C Beauchene et al. Tranexamic Acid as First-Line Emergency Treatment for Episodes of Bradykinin-Mediated Angioedema Induced by ACE Inhibitors. Rev Med Interne. 2018 Oct;39(10):772-776.

Moellman, J.J., Bernstein, J.A., Lindsell, C., Banerji, A., Busse, P.J., Camargo, C.A., Collins, S.P., Craig, T.J., Lumry, W.R., Nowak, R., Pines, J.M., Raja, A.S., Riedl, M., Ward, M.J., Zuraw, B.L., Diercks, D., Hiestand, B., Campbell, R.L., Schneider, S. and Sinert, R. (2014) ‘A consensus parameter for the evaluation and management of Angioedema in the emergency department’, Academic Emergency Medicine, 21(4), pp. 469–484.

Craig TJ, Levy RJ, Wasserman RL, et al. Efficacy of human C1 esterase inhibitor concentrate compared with placebo in acute hereditary angioedema attacks. J Allergy Clin Immunol. 2009; 124(4):801.

Bas M, Greve J, Stelter K, et al. Therapeutic efficacy of icatibant in angioedema induced by angiotensin-converting enzyme inhibitors: a case series. Ann Emerg Med. 2010; 56(3):278-282.

C Beauchene et al. Tranexamic Acid as First-Line Emergency Treatment for Episodes of Bradykinin-Mediated Angioedema Induced by ACE Inhibitors. Rev Med Interne. 2018 Oct;39(10):772-776.

Baş M, Greve J, Stelter K, et al. A randomized trial of icatibant in ACE-inhibitor-induced angioedema. N Engl J Med. 2015; 372(5):418-25.

Bernstein JA, Moellman JJ, Collins SP, et al. Effectiveness of ecallantide in treating angiotensin-converting enzyme inhibitor-induced angioedema in the emergency department. Ann Allergy Asthma Immunol. 2015; 114(3):245.

Lewis, L.M., Graffeo, C., Crosley, P., Klausner, H.A., Clark, C.L., Frank, A., Miner, J., Iarrobino, R. and Chyung, Y. (2015) ‘Ecallantide for the acute treatment of angiotensin-converting enzyme Inhibitor–Induced Angioedema: A Multicenter, Randomized, controlled trial’, Annals of Emergency Medicine, 65(2), pp. 204–213.

Ishoo E, et al. Predicting airway risk in angioedema: staging system based on presentation. Otolaryngol Head Neck Surg. 1999; 121(3):263-268.