The molecular, and in hereditary forms, the genetic basis of angioedema is becoming better understood, leading to improved management including more focussed emergency room diagnosis and treatment.
It is now possible to distinguish in the laboratory the three subtypes of hereditary angioedema from each other and from acquired C′1 esterase inhibitor deficiency and this is important because of the impact on treatment.
C′1 inhibitor concentrate is established as a safe and effective emergency room treatment for hereditary C′1 inhibitor deficient angioedema, but in the acquired forms, because of increased consumption, it may have to be supplemented by tranexamic acid treatment. Icatibant, a bradykinin antagonist, has now been licensed in Europe for acute attacks of types 1 and 2 hereditary angioedema. Both Icatibant and the kallikrein inhibitor ecallantide are promising treatments for ACE inhibitor angioedema though not yet licensed for this indication.
In all cases of acute angioedema whatever the type or cause, the cornerstones of emergency treatment include securing the airway, establishing an intravenous line and parenteral adrenaline. Admission for 24h observation is also advisable for all but the mildest cases.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Kaplan AP, Greaves MW. Angioedema. J Am Acad Dermatol. 2005; 53: 373–388.
Champion RH, Roberts SOB, Carpenter RG, Roger JH. Urticaria and angioedema: a review of 554 patients. Br J Dermatol. 1969; 81: 588–597.
Kaplan AP, Greaves MW. Angioedema. Current Allergy Clin Immunol. 2006; 19: 104–111.
Zuberbier T, Bindslev-Jensen C, Canonica W, et al. EAACI/GA2LEN/EDF guideline: management of urticaria. Allergy. 2006; 61: 321–331.
Downing A, Jacobsen J, Sorenesen HT, et al. Risk of hospitalisation for angioedema among users of newer Cox – 2 selective inhibitors and other nonsteroid anti-inflammatory drugs. Br J Clin Pharmacol. 2006; 62: 496–501.
Wakelin SH, White IR. Latex allergy. Clin Exp Dermatol. 1999; 24: 245–248.
Sabroe RA, Seed PT, Francis DM, et al., Chronic idiopathic urticaria: comparison of clinical features of patients with or without anti-FceR1 or anti-IgE autoantibodies. J Am Acad Dermatol. 1999; 40: 443–450.
Kozel MA, Mekkes JR, Bossuyt PM, Bos JD. Natural course of physical and chronic urticaria and angioedema in 220 patients. J Am Acad Dermatol. 2001; 45: 387–391.
Hide M, Francis DM, Grattan C, et al. Autoantibodies against the high-affinity IgE receptor as a cause for histamine release in chronic urticaria. New Engl J Med. 1993; 328: 1599–1604.
Sabroe RA, Greaves MW. Chronic idiopathic urticaria with functional autoantibodies: 12 years on. Br J Dermatol. 2006; 154: 813–819.
Greaves MW. Chronic idiopathic urticaria. Curr Opin Allergy Clin Immunol. 2003; 3: 363–368.
Nimii N, Francis DM, Kermani F, et al. Dermal mast cell activation by autoantibodies against the high affinity IgE receptor in chronic urticaria. J Invest Dermatol. 1996; 106: 1001–1006.
Wedi B, Novacovic V, Koerner M, et al. Chronic urticaria serum induces histamine release, leukotriene and basophil CD 63 surface expression-inhibitory effects of anti-inflammatory drugs. J Allergy Clin Immunol. 2000; 105: 552–560.
Confino-Cohen R, Aharoni D, Goldberg A, et al. Evidence for aberration of regulation of the p21 Ras pathway in PBMC's of patients with chronic idiopathic urticaria. J Allergy Clin Immunol. 2002; 109: 349–356.
Black AK. Physical urticaria and cholinergic urticaria. In Greaves MW, Kaplan AP, eds, Urticaria and Angioedema. New York: Marcel Dekker, 2004: 171–214.
Wisnieski JJ, Baer AN, Christensen J, et al. Hypocomplementaemic urticarial vasculitis syndrome. Clinical and serologic findings in 8 patients. Medicine (Baltimore) 1995; 74: 24–41.
Osler W. Hereditary angioneurotic edema. Am J Med Sci. 1888; 95: 362–367.
Nussberger J, Cugno M, Amstutz C, et al. Plasma bradykinin in angioedema. Lancet. 1998; 351: 1693–1697.
Visy B, Fust G, Varga L, et al. Sex hormones in hereditary angioneurotic oedema. Clin Endocrinol. (Oxf) 2004; 60: 508–515.
Yip J, Cunliffe WJ. Hormonally exacerbated Hereditary angioedoema. Australas. J Dermatol. 1992; 33: 35–38.
Gompels MM, Lock RJ, Morgan JE, et al. A multicentre study of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency. J Clin Pathol. 2002; 55: 145–147.
Tosi M. Molecular genetics of C'1 inhibitor. Immunobiology 1998; 119: 358–365.
Kramer J, Rosen F, Colten H, et al. Transinhibition of C'1 inhibitor synthesis in type I hereditary angioedema. J Clin Immunol. 1993; 91: 1258–1262.
Farkas H, Harmat G, Fust G, et al. Clinical management of hereditary angio-oedema in children. Pediatr Allergy Immunol 2002; 13: 153–161.
Frank MM, Gelfand JA, Atkinson JP. Hereditary angioedema: the clinical syndrome and its management. Ann Int Med. 1976; 84: 580–593.
Farkas H, Harmat G, Fay A, et al. erythema marginatum preceding an acute oedematous attack of hereditary angio-neurotic oedema. Acta Derm Venereol. 2001; 81: 376–377.
Shah TJ, Knowles WO, Mc Geadt SJ. Hereditary angioedema with recurrent abdominal pain and ascites. J Allergy Clin Immunol. 1995; 96: 259–261.
Farkas H, Harmat G, Kaposi PN, et al. Ultrasonography in the diagnosis and monitoring of ascites in acute abdominal attacks of hereditary angioneurotic oedema. Eur J Gastroenterol Hepatol. 2001; 13: 1225–1230.
Bork K, Siedlecki K, Bosch S, Schopf RE, Kreuz, W. Asphyxiation due to laryngeal edema in patients with hereditary angioedema. Mayo Clin Proc. 2000; 75: 349–354.
Koide M, Shirahama S, Tokura Y, et al. Lupus erythematosus associated with C1 inhibitor deficiency. J Dermatol. 2002; 29: 503–507.
Gompels, MM, Lock RJ, Unsworth GJ, et al. Misdiagnosis of hereditary angioedema type 1 and type 2. Br J Dermatol. 2003; 148: 719–723.
Zuraw BL, Sugimoto S, Curd JG. The value of rocket immunoelectrophoresis for C'4 activation in the evaluation of patients with angioedema or C1-inhibitor deficiency. J Allergy Clin Immunol. 1986; 78: 1115–1120.
Nielson EW, Johanson HT, Holt J, Mollines TE. C1 inhibitor and diagnosis of HAE in new-borns. Paediatr Res. 1994; 35: 184–187.
Warin RP, Cunliffe WJ, Greaves MW, Wallington TB. Recurrent angioedema: familial and oestrogen-induced. Br J Dermatol. 1986; 115: 731–734.
Bork K, Barnstedt SE, Koch P, Traupe H. Hereditary angioedema with normal C1-inhibitor activity in women. Lancet. 2000; 356: 213–217.
Martin L, Degenne D, Toutain A, Ponard D, Watier H. Hereditary angioedema type 111: an additional French pedigree with autosomal dominant transmission. J Allergy Clin Immunol. 2001; 107: 747–748.
Binkley KE, Davis AE. Estrogen-dependent inherited angioedema. Transfus Apheresis Sci. 2003; 29: 215–219.
Dewald G, Bork K. Missense mutations in the coaulation factor XII (Hageman factor) genein hereditary angioedema with normal C′ 1 inhibitor. Biochem. Biophys. Res. Commun. 2006; 343: 1286–1289.
Schreiber A, Zweiman B, Atkins P, et al. Acquired angioedema with lymphoproliferative disorder: association of C′1 inhibitor deficiency with cellular abnormality. Blood. 1976; 48: 567–580.
Geha R, Quinti I, Austen KF, et al. Acquired C′1 inhibitor deficiency associated with anti-idiotypic antibody to monoclonal immunoglobulins. N Engl J Med. 1985; 312: 534–540.
Donaldson V, Hess E, McAdams A. Lupus erythematosus-like disease in three unrelated women with hereditary angioneurotic edema. Ann Int Med. 1977; 86: 312–313.
Jackson J, Sim R, Whelan A, et al. An IgG autoantibody which inactivates C1-inhibitor. Nature. 1986; 323: 722–724.
Alsenz, J, Bork K, Loos M. Autoantibody-mediated acquired deficiency of C1 Inhibitor. N Engl J Med. 1987; 316: 1360–1366.
Chevailler A, Arlaud G, Ponard D, et al. C1-inhibitor binding monoclonal immunoglobulins in three patients with acquired angioneurotic oedema. J Allergy Clin Immunol. 1996; 97: 998–1008.
Agah R, Bandi V, Gunturpalli KK. Angioedema: the role ofACE inhibitors and factors associated with poor outcome. Intensive Care Med. 1999; 23: 793–796.
Hedner T, Samuelsson O, Lunde H et al. Angioedema in relation to treatment with angiotensin converting enzyme inhibitors. Brit Med. J. 1992; 304: 941–946.
Lin RY, Shah SN, Karandish K. Increasing Hospitalisations due to angioedema in the united States. J Allergy Clin Immunol. 2008; Suppl. 1; 121: S99 (abs).
Pigman EC, Scott JL. Angioedema in the emergency department: the impact of angiotensin converting enzyme inhibitors. Am J Emerg Med. 1993; 11: 350–354.
Schiller PI, Langauer MS, Haefeli WE, et al. Angiotensin-converting enzyme inhibitor induced angioedema: late onset, irregular course and potential role of triggers. Allergy. 1997; 52: 432–435.
Brown NJ, Snowden M, Griffin MR. Recurrent angiotensin convertase enzyme inhibitor-associated angioedema. J Am Med Assoc. 1997; 278: 232–233.
Greaves MW, Shuster S. Responses of skin blood vessels to bradykinin, histamine and 5-hydroxytryptamine. J Physiol. 1967; 193: 255–267.
Nussberger J, Cugno M, Amstutz C, et al. Plasma bradykinin in angioedema. Lancet 1998; 351: 1693–1697.
Mathews KP, Pan M, Gardner MJ, Hugli T. Familial carboxypeptidase N deficiency. Ann Int Med. 1980; 93: 443–445.
Sabroe RA, Black AK. Angiotensin converting enzyme (ACE) inhibitors and angioedema. Br J Dermatol. 1997; 136: 153–158.
Irons BK, Kumar A. Valsartan-induced angioedema. Ann Pharmacother. 2003; 37: 1024–1027.
Abdi R, Dong VM, Lee CJ, Ntoso KA. Angiotensin 11 receptor blocker-associated angioedema: on the heels of ACE inhibitor angioedema. Pharmacotherapy. 2002; 22: 1173–1175.
Tan Y, Hutchinson FN, Jaffa AA. Mechanisms of angiotensin 11-induced expression of B2 kinin receptors. Am J Physiol Heart Circ Physiol. 2004; 286: H926–932.
Coats AJ. Omipatrilat — the story of Overture and Octave. Int J Cardiol. 2002; 86: 1–4.
Pickering RJ, Kelly JR, Good RA, Gerwuz H. Replacement therapy in hereditary angioedema: successful treatment of two patients with fresh frozen plasma. Lancet. 1969; 1: 326–330.
Gompels MM, Lock RJ. C1 inhibitor deficiency: management. Clin Exp Dermatol. 2005; 30: 737–740.
Waytes TA, Rosen FS, Frank MM. Treatment of hereditary angioedema with a vapor-heated C′1 inhibitor concentrate. N Engl J Med. 1996; 334: 1630–1634.
Bowen T, Cicardi M, Farkas H, et al. Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema. J Allergy Clin Immunol. 2004; 114: 629–646.
Bork K, Yasothan U, Kirkpatrick P. Icatibant. Nat Rev Drug Disc 2008; 7: 801–802.
Cugno M, Cicardi M, Agostoni A. Activation of the contact system and fibrinolysis in autoimmune acquired angioedema: a rationale for prophylactic use of tranexamic acid. J Allergy Clin Immunol. 1994; 93: 870–876.
Oike Y, Ogata Y, Higashi D, et al. Fatal angioedema associated with enalapril. Int Med. 1993; 32: 308–310.
Thompson T, Frable M. Drug-induced life-threatening angioedema revisited. Laryngoscope. 1993; 103: 10–12.
Orfan, N, Patterson R, Dykewicz M. Severe angioedema related to ACE inhibitors in patients with a history of idiopathic angioedema. JAMA. 1990; 264: 1287–1289.
Kaplan AP. Clinical practice. Chronic urticaria and angioedema. N Engl J Med. 2002; 346: 175–179.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2009 Springer
About this chapter
Cite this chapter
Greaves, M.W., Kaplan, A.P. (2009). Angioedema in the Emergency Department. In: Pawankar, R., Holgate, S.T., Rosenwasser, L.J. (eds) Allergy Frontiers: Diagnosis and Health Economics. Allergy Frontiers, vol 4. Springer, Tokyo. https://doi.org/10.1007/978-4-431-98349-1_19
Download citation
DOI: https://doi.org/10.1007/978-4-431-98349-1_19
Publisher Name: Springer, Tokyo
Print ISBN: 978-4-431-98293-7
Online ISBN: 978-4-431-98349-1
eBook Packages: MedicineMedicine (R0)