eMedicine Specialties > Dermatology > Allergy & Immunology

Angioedema, Acquired: Follow-up

Author: Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Coauthor(s): Amanda Carolfi, University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School
Contributor Information and Disclosures

Updated: Aug 18, 2009

Follow-up

Prognosis

  • The prognosis for acquired angioedema is variable, and, in most patients, it depends on control of the underlying disorder. However, even with appropriate treatment of the underlying disease, patients may only be free of symptoms temporarily. Additionally, some patients who have had their underlying disease treated will be free of symptoms related to angioedema but will continue to have biochemical abnormalities.7
  • Compared with the general population, patients with acquired angioedema have a higher incidence of B-cell malignancies.
  • Patients with acquired angioedema and a concurrent diagnosis of monoclonal gammopathy of undetermined significance (MGUS) do not have an increased risk for progression to malignancy compared with patients with a sole diagnosis of MGUS.

Patient Education

Miscellaneous

Special Concerns

  • Tranexamic acid may be used during pregnancy.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Kathleen M. Rossy, MD, and previous Chief Editor, William D. James, MD, to the development and writing of this article.



More on Angioedema, Acquired

Overview: Angioedema, Acquired
Differential Diagnoses & Workup: Angioedema, Acquired
Treatment & Medication: Angioedema, Acquired
Follow-up: Angioedema, Acquired
References

References

  1. 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].

  2. 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].

  3. 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].

  4. 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].

  5. Leiden, The Netherlands. Biotech company Pharming Group NV ("Pharming" or "the Company") (NYSE Euronext: PHARM) today announces that, in agreement with the European Medicines Agency (EMEA), the dossier for the European Marketing Authorisation Application (MAA) of Rhucin(R) will be submitted in September 2009. CheckOrphan. Available at http://www.checkorphan.org/news/pharming_confirms_rhucin_european_maa_filing_timeline. Accessed 8/5/2009.

  6. Craig T, Riedl M, Dykewicz MS, et al. When is prophylaxis for hereditary angioedema necessary?. Ann Allergy Asthma Immunol. May 2009;102(5):366-72. [Medline].

  7. Banerji A, Sheffer AL. The spectrum of chronic angioedema. Allergy Asthma Proc. Jan-Feb 2009;30(1):11-6. [Medline].

  8. Agostoni A, Aygoren-Pursun E, Binkley KE, et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol. Sep 2004;114(3 Suppl):S51-131. [Medline].

  9. Alsenz J, Bork K, Loos M. Autoantibody-mediated acquired deficiency of C1 inhibitor. N Engl J Med. May 28 1987;316(22):1360-6. [Medline].

  10. Cicardi M, Zingale LC, Pappalardo E, Folcioni A, Agostoni A. Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies. Medicine (Baltimore). Jul 2003;82(4):274-81. [Medline].

  11. Dobson G, Edgar D, Trinder J. Angioedema of the tongue due to acquired C1 esterase inhibitor deficiency. Anaesth Intensive Care. Feb 2003;31(1):99-102. [Medline].

  12. Donaldson VH, Bernstein DI, Wagner CJ, Mitchell BH, Scinto J, Bernstein IL. Angioneurotic edema with acquired C1- inhibitor deficiency and autoantibody to C1- inhibitor: response to plasmapheresis and cytotoxic therapy. J Lab Clin Med. Apr 1992;119(4):397-406. [Medline].

  13. Fremeaux-Bacchi V, Guinnepain MT, Cacoub P, et al. Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2. Am J Med. Aug 15 2002;113(3):194-9. [Medline].

  14. Gaur S, Cooley J, Aish L, Weinstein R. Lymphoma-associated paraneoplastic angioedema with normal C1-inhibitor activity: does danazol work?. Am J Hematol. Nov 2004;77(3):296-8. [Medline].

  15. Grace RJ, Jacob A, Mainwaring CJ, McVerry BA. Acquired C1 esterase inhibitor deficiency as manifestation of T-cell lymphoproliferative disorder. Lancet. Jul 14 1990;336(8707):118. [Medline].

  16. Harrison NK, Twelves C, Addis BJ, Taylor AJ, Souhami RL, Isaacson PG. Peripheral T-cell lymphoma presenting with angioedema and diffuse pulmonary infiltrates. Am Rev Respir Dis. Oct 1988;138(4):976-80. [Medline].

  17. Hentges F, Kohnen M, Grigioni F, Reichert P, Humbel R, Schneider F. Production and characterization of monoclonal antibodies directed against high and low molecular weight kininogens. Bull Soc Sci Med Grand Duche Luxemb. 1994;131(2):9-17. [Medline].

  18. Heymann WR. Acquired angioedema. J Am Acad Dermatol. Apr 1997;36(4):611-5. [Medline].

  19. Kaplan AP, Greaves MW. Angioedema. J Am Acad Dermatol. Sep 2005;53(3):373-88; quiz 389-92. [Medline].

  20. Sinclair D, Smith A, Cranfield T, Lock RJ. Acquired C1 esterase inhibitor deficiency or serendipity? The chance finding of a paraprotein after an apparently low C1 esterase inhibitor concentration. J Clin Pathol. Apr 2004;57(4):445-7. [Medline].

  21. Valsecchi R, Reseghetti A, Pansera B, Di Landro A. Autoimmune C1 inhibitor deficiency and angioedema. Dermatology. 1997;195(2):169-72. [Medline].

  22. Varvarovska J, Sykora J, Stozicky F, Chytra I. Acquired angioedema and Helicobacter pylori infection in a child. Eur J Pediatr. Oct 2003;162(10):707-9. [Medline].

  23. Wellwood J, Taylor K, Wright S, Bentley M, Eliadis P. Angioedema in the emergency department: a presentation of lymphoma. Emerg Med (Fremantle). Dec 2001;13(4):465-8. [Medline].

  24. Wong DT, Gadsden JC. Acute upper airway angioedema secondary to acquired C1 esterase inhibitor deficiency: a case report. Can J Anaesth. Nov 2003;50(9):900-3. [Medline].

  25. Zingale LC, Castelli R, Zanichelli A, Cicardi M. Acquired deficiency of the inhibitor of the first complement component: presentation, diagnosis, course, and conventional management. Immunol Allergy Clin North Am. Nov 2006;26(4):669-90. [Medline].

  26. Zuraw BL, Altman LC. Acute consumption of C1 inhibitor in a patient with acquired C1-inhibitor deficiency syndrome. J Allergy Clin Immunol. Dec 1991;88(6):908-18. [Medline].

Further Reading

Keywords

angioedema, acquired angioedema, AAE, Caldwell syndrome, acquired angioedema

Contributor Information and Disclosures

Author

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Amanda Carolfi, University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School
Amanda Carolfi is a member of the following medical societies: American Medical Association, American Medical Student Association/Foundation, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Paul Krusinski, MD, Director of Dermatology, Professor, Department of Internal Medicine, Fletcher Allen Health Care, University of Vermont
Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.