Acquired Angioedema Treatment & Management
- Author: Ru'aa Al Harithy, MBBS, FRCPC; Chief Editor: William D James, MD more...
Approach Considerations
A variety of agents can be used for acute attacks of acquired angioedema (see Medication). Depending on the symptoms and the site of the angioedema, intensive support may be necessary, including intravenous fluids. Intubation may be necessary in cases of laryngeal edema.
When possible, the underlying disorder should be treated. The resolution of angioedema has been reported with the treatment of underlying disease, although recurrences have occurred despite appropriate treatment of the disorder. In acquired angioedema type I (AAE-I), treatment of the associated lymphoproliferative process may result in correction of the abnormality.
Go to Angioedema, Pediatric Angioedema, Emergent Treatment of Angioedema, and Hereditary Angioedema for complete information on this topic.
Prophylaxis
Two papers have reported effective treatment of acquired angioedema cases with a series of 4 weekly injections with rituximab (a chimeric monoclonal antibody to CD20). In one study, rituximab treatment resulted in normalization of C1-INH and C4 levels and long-term remission of angioedema attacks in 3 patients with severe acquired angioedema.[8] In a second study, a patient with acquired angioedema type II refractory to standard treatment experienced a 6-month attack-free interval after treatment with rituximab.[9]
Another immunosuppressant drug, etanercept, was reported to control angioedema in a 57-year old man with psoriatic arthritis. This man was treated with 25 mg of etanercept administered subcutaneously twice per week for recalcitrant psoriatic arthritis. The treatment also resulted in improvement of the patient's angioedema. The authors hypothesized that etanercept may have improved the angioedema by decreasing inflammation and vascular permeability.[10]
Caldwell JR, Ruddy S, Schur PH, Austen KF. Acquired C1 inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol. 1972;1:39-52.
Caballero T, Baeza ML, Cabañas R, et al. Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part I. Classification, epidemiology, pathophysiology, genetics, clinical symptoms, and diagnosis. J Investig Allergol Clin Immunol. 2011;21(5):333-47; quiz follow 347. [Medline].
Cugno M, Zanichelli A, Foieni F, Caccia S, Cicardi M. C1-inhibitor deficiency and angioedema: molecular mechanisms and clinical progress. Trends Mol Med. Feb 2009;15(2):69-78. [Medline].
Cugno M, Castelli R, Cicardi M. Angioedema due to acquired C1-inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation. Autoimmun Rev. Dec 2008;8(2):156-9. [Medline].
Banerji A, Sheffer AL. The spectrum of chronic angioedema. Allergy Asthma Proc. Jan-Feb 2009;30(1):11-6. [Medline].
Cicardi M, Zanichelli A. Acquired angioedema. Allergy Asthma Clin Immunol. Jul 28 2010;6(1):14. [Medline]. [Full Text].
Bouillet-Claveyrolas L, Ponard D, Drouet C, Massot C. Clinical and biological distinctions between type I and type II acquired angioedema. Am J Med. Oct 1 2003;115(5):420-1. [Medline].
Levi M, Hack CE, van Oers MH. Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. Am J Med. Aug 2006;119(8):e3-5. [Medline].
Ziakas PD, Giannouli S, Psimenou E, Evangelia K, Tzioufas AG, Voulgarelis M. Acquired angioedema: a new target for rituximab?. Haematologica. Aug 2004;89(8):ELT13. [Medline].
Rottem M, Mader R. Successful use of etanercept in acquired angioedema in a patient with psoriatic arthritis. J Rheumatol. Jan 2010;37(1):209. [Medline].
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