Angioedema: Difference between revisions
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==Background== | ==Background== | ||
*Angioedema is paroxysmal, nondemarcated swelling of dermal or submucosal layers of skin or mucosa | *Angioedema is paroxysmal, nondemarcated swelling of dermal or submucosal layers of skin or mucosa | ||
**Swelling is asymmetric, nonpitting, and nonpruritic | **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 | |||
[[File:Nejmicm020617 f1.jpg|thumb|Uvular Angioedema (Quincke's Disease)]] | |||
===Etiologies=== | ===Etiologies=== | ||
*Congenital or acquired loss of C1 esterase inhibitor | *'''Allergic angioedema:''' [[Allergic reaction|IgE–mediated type I]] [[Hypersensitivity Reaction|hypersensitivity reaction]] | ||
* | *'''Hereditary angioedema:''' Congenital or acquired loss of C1 esterase inhibitor | ||
*ACEI adverse reaction | **Due to C1 esterase inhibitor deficiency | ||
*Idiopathic | **Leads to unregulated activity of vasoactive mediators (bradykinin) associated with complement pathway | ||
**Autosomal dominant | |||
**Edema of face, extremities, bowel wall | |||
*'''[[ACE inhibitor|ACE-I]] induced angioedema:''' ACEI adverse reaction from excessive bradykinin | |||
*'''Idiopathic angioedema''' | |||
==Clinical Features== | |||
[[File:Angioedema2010.jpg|thumb|Angioedema of face.]] | |||
[[File:Angioedema_post_lisinopril_use_2014-12-08_12-35.jpg|thumbnail|Angioedema]] | [[File:Angioedema_post_lisinopril_use_2014-12-08_12-35.jpg|thumbnail|Angioedema]] | ||
[[File:Angioedema2013.jpg|thumbnail|Angioedema of tongue]] | [[File:Angioedema2013.jpg|thumbnail|Angioedema of tongue]] | ||
*Angioedema | |||
*Swelling most often includes face, lips, and/or tongue | |||
*May also involve<ref>https://www.merckmanuals.com/professional/immunology-allergic-disorders/allergic,-autoimmune,-and-other-hypersensitivity-disorders/angioedema?query=angioedema</ref>: | |||
**Upper airway--> [[respiratory distress]], [[stridor]] | |||
**Face, hands, genitals | |||
**Intestines--> [[nausea/vomiting]], [[abdominal pain]], [[diarrhea]], SBO-like presentation | |||
== | ==Differential Diagnosis== | ||
{{Template:Acute Allergic DDX}} | |||
=== | ==Evaluation== | ||
===Hereditary angioedema=== | |||
*Suspect in patients with history of recurrent peripheral angioedema and abdominal pain | *Suspect in patients with history of recurrent peripheral angioedema and abdominal pain | ||
**75% experience onset of symptoms before age 15yr | **75% experience onset of symptoms before age 15yr | ||
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*Decreased levels of C1 and C4 esterase inhibitors confirms diagnosis | *Decreased levels of C1 and C4 esterase inhibitors confirms diagnosis | ||
= | ===ACE inhibitor-induced angioedema=== | ||
==ACE inhibitor-induced angioedema | |||
*Incidence is highest within the first month; however, may occur at anytime | *Incidence is highest within the first month; however, may occur at anytime | ||
*40% present months to years after initial dose<ref>Winters ME, et al. Emergency department management of patients with ACE-inhibitor angioedema. JEM. 2013; 45(5):775–780.</ref> | *40% present months to years after initial dose<ref>Winters ME, et al. Emergency department management of patients with ACE-inhibitor angioedema. JEM. 2013; 45(5):775–780.</ref> | ||
Line 40: | Line 42: | ||
*Physiology more closely related to bradykinin-mediated pathway than IgE-mediated pathway, therefore current treatments may be insufficient | *Physiology more closely related to bradykinin-mediated pathway than IgE-mediated pathway, therefore current treatments may be insufficient | ||
=== | ==Management== | ||
* | ===General=== | ||
*Consider [[Epinephrine]] 0.3mg IM if there is any concern this could be allergic in nature | |||
*Consider | *Consider [[Glucagon]] 1-5mg IV if patient is on beta-blockers and not responding to Epinephrine | ||
* | *Consider [[tranxemic acid]] (1 g IV, over 10 min) for bradykinin-mediated angioedema <ref>[https://emcrit.org/ibcc/angioedema/#algorithms IBCC Angioedema Chapter Algorithm]</ref><ref>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.</ref> | ||
* | *[[FFP]] for possible etiology related to bradykinin or C1-esterase deficiency<ref>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.</ref> | ||
* | **2 units | ||
*Consider definitive airway if voice change, hoarseness, stridor, dyspnea | |||
**Prepare for a [[difficult airway]] which can include need for fiberoptics, ENT/anesthesia assistance, [[surgical airway]], or transfer to the OR | |||
== | ===Hereditary Angioedema=== | ||
====First-Line Therapies==== | |||
*[[C1 inhibitor]] (C1INH)<ref>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.</ref> | |||
**1000 units if ≤50kg | |||
**1500 units if >50-75kg | |||
**2000 units if >75-100kg | |||
**2500 units if >100kg | |||
*[[Ecallantide]] | |||
**10mg SQ x 3 in different anatomical locations (30mg in total) | |||
*[[Icatibant]] | |||
**30mg SQ | |||
===ACE-I Induced Angioedema=== | |||
*Typical anaphylaxis medications do not effect bradykinin levels<ref>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.</ref>, but consider: | |||
**[[Epinephrine]] 0.3mg IM | |||
**[[Diphenhydramine]] 50mg IV | |||
**[[Methylprednisolone]] 125mg IV | |||
*[[Icatibant]] | |||
**30mg SQ | |||
**Significantly decreases time to complete resolution (8 hrs vs 27.1 hrs)<ref>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.</ref> | |||
**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<ref>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.</ref> | |||
**No benefit seen in mild-moderate cases in multi-center, double blind study<ref>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.</ref> | |||
==Disposition== | ==Disposition== | ||
*Consider discharge after 4-6 hrs | *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 | *24 hrs obs if epinephrine given | ||
===Ishoo Staging^<ref>Ishoo E, et al. Predicting airway risk in angioedema: staging system based on presentation. Otolaryngol Head Neck Surg. 1999; 121(3):263-268.</ref>=== | |||
{| {{table}} | |||
| align="center" style="background:#f0f0f0;"|'''Stage''' | |||
| align="center" style="background:#f0f0f0;"|'''Area''' | |||
| align="center" style="background:#f0f0f0;"|'''Outpatient''' | |||
| align="center" style="background:#f0f0f0;"|'''Floor''' | |||
| align="center" style="background:#f0f0f0;"|'''ICU''' | |||
| align="center" style="background:#f0f0f0;"|'''Advanced Airway''' | |||
|- | |||
| 1||Face/lip||48%||52%||0%||0% | |||
|- | |||
| 2||Soft palate||60%||40%||0%||0% | |||
|- | |||
| 3||Tongue||26%||7%||67%||7% | |||
|- | |||
| 4||Larynx||0%||0%||100%||24% | |||
|} | |||
^Based on retrospective study | |||
==See Also== | ==See Also== |
Revision as of 02:18, 12 June 2020
Background
- 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
Etiologies
- Allergic angioedema: IgE–mediated type I hypersensitivity reaction
- 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
- Edema of face, extremities, bowel wall
- ACE-I induced angioedema: ACEI adverse reaction from excessive bradykinin
- Idiopathic angioedema
Clinical Features
- Angioedema
- Swelling most often includes face, lips, and/or tongue
- May also involve[1]:
- Upper airway--> respiratory distress, stridor
- Face, hands, genitals
- Intestines--> nausea/vomiting, abdominal pain, diarrhea, SBO-like presentation
Differential Diagnosis
Acute allergic reaction
- Allergic reaction/urticaria
- Anaphylaxis
- Angioedema
- Anxiety attack
- Asthma exacerbation
- Carcinoid syndrome
- Cold urticaria
- Contrast induced allergic reaction
- Scombroid
- Shock
- Transfusion reaction
Evaluation
Hereditary angioedema
- 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
ACE inhibitor-induced angioedema
- Incidence is highest within the first month; however, may occur at anytime
- 40% present months to years after initial dose[2]
- 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
Management
General
- Consider Epinephrine 0.3mg IM if there is any concern this could be allergic in nature
- Consider Glucagon 1-5mg IV if patient is on beta-blockers and not responding to Epinephrine
- Consider tranxemic acid (1 g IV, over 10 min) for bradykinin-mediated angioedema [3][4]
- FFP for possible etiology related to bradykinin or C1-esterase deficiency[5]
- 2 units
- Consider definitive airway if voice change, hoarseness, stridor, dyspnea
- Prepare for a difficult airway which can include need for fiberoptics, ENT/anesthesia assistance, surgical airway, or transfer to the OR
Hereditary Angioedema
First-Line Therapies
- C1 inhibitor (C1INH)[6]
- 1000 units if ≤50kg
- 1500 units if >50-75kg
- 2000 units if >75-100kg
- 2500 units if >100kg
- Ecallantide
- 10mg SQ x 3 in different anatomical locations (30mg in total)
- Icatibant
- 30mg SQ
ACE-I Induced Angioedema
- Typical anaphylaxis medications do not effect bradykinin levels[7], but consider:
- Epinephrine 0.3mg IM
- Diphenhydramine 50mg IV
- Methylprednisolone 125mg IV
- Icatibant
- 30mg SQ
- Significantly decreases time to complete resolution (8 hrs vs 27.1 hrs)[8]
- Note: control group did not receive FFP
- Consider Ecallantide
Disposition
- 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^[11]
Stage | Area | Outpatient | Floor | ICU | Advanced Airway |
1 | Face/lip | 48% | 52% | 0% | 0% |
2 | Soft palate | 60% | 40% | 0% | 0% |
3 | Tongue | 26% | 7% | 67% | 7% |
4 | Larynx | 0% | 0% | 100% | 24% |
^Based on retrospective study
See Also
References
- ↑ 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.
- ↑ IBCC Angioedema Chapter Algorithm
- ↑ 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.
- ↑ 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.