eMedicine Specialties > Allergy and Immunology > Complement-Related Abnormalities

Hereditary Angioedema: Follow-up

Author: Michael M Frank, MD, Distinguished Professor, Departments of Pediatrics and Medicine, Professor, Pediatric Allergy and Immunology Division, Duke University School of Medicine
Contributor Information and Disclosures

Updated: Oct 26, 2009

Follow-up

Further Inpatient Care

  • Inpatient care for patients with angioedema is based on providing patient support during an attack. Protection of the airway is of foremost concern and should be handled in cooperation with physicians capable of performing emergent intubation or tracheostomy, if required. Future use of C1 inhibitor (C1-INH) concentrate may provide another option in the emergent care of these patients.
  • Once recovery is achieved, proper referral for education and long-term management should be arranged.

Further Outpatient Care

  • Outpatient management should focus on prophylaxis and patient education.

Inpatient & Outpatient Medications

  • Preventative therapy with attenuated androgens is currently the initial mode of treatment. Therapy should be minimized, balancing disease severity with minimizing adverse effects. The specific drug most commonly used is danazol. All attenuated androgens are useful in treatment.
  • A discussion of future plans, such as pregnancy, should be routine. The positive and negative aspects of androgen therapy should be discussed openly.
  • Anticipatory guidance with respect to oral surgery or any major procedure that will involve airway instrumentation should be stressed during follow-up care.

Complications

  • Airway obstruction can complicate episodes of laryngeal edema.

Prognosis

  • Prognosis has improved over the past 20 years, with fewer adverse effects, both short-term and long-term, from judicious use of androgens. The addition of C1-INH concentrate will greatly enhance the care of these patients.

Patient Education

  • Patients should be educated about possible triggering factors of their attacks. They should also be advised of the autosomal dominant inheritance pattern of hereditary angioedema (HAE) and that they should anticipate that 50% of their children will be affected. However, phenotypic expression of the condition may vary significantly within families.

Miscellaneous

Medicolegal Pitfalls

  • Hereditary angioedema (HAE) is a treatable disease; however, the agents used to treat more common forms of angioedema are not effective. The diagnosis of HAE should be considered when angioedema is not associated with urticaria or when cutaneous or laryngeal attacks do not respond to the usual therapy. Once the diagnosis has been made, efforts should be made to prevent attacks associated with dental and surgical procedures.

Special Concerns

  • Other types of angioedema without urticaria, referred to as acquired angioedema (AAE), can complicate the process of diagnostic evaluation. As with patients with HAE, those with AAE have decreased C1 inhibitor (C1-INH) activity and experience the same spectrum of clinical disease. However, the 2 conditions differ in several respects. Acquired angioedema is due to (1) an autoantibody to the C1-INH that prevents its function, (2) marked utilization of the normal C1 inhibitor by high levels of antigen-antibody complexes, or (3) factors formed by lymphoid tumors that destroy C1-INH activity. In general, these patients have low levels of C1q, which distinguishes them from patients with HAE, who have normal levels of the protein.
 


More on Hereditary Angioedema

Overview: Hereditary Angioedema
Differential Diagnoses & Workup: Hereditary Angioedema
Treatment & Medication: Hereditary Angioedema
Follow-up: Hereditary Angioedema
References
[ CLOSE WINDOW ]

References

  1. Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med. Sep 4 2008;359(10):1027-36. [Medline].

  2. Frank MM, Gelfand JA, Atkinson JP. Hereditary angioedema: the clinical syndrome and its management. Ann Intern Med. May 1976;84(5):580-93. [Medline].

  3. Rosen FS, Alper CA, Pensky J, et al. Genetically determined heterogeneity of the C1 esterase inhibitor in patients with hereditary angioneurotic edema. J Clin Invest. Oct 1971;50(10):2143-9. [Medline].

  4. Bork K, Barnstedt SE, Koch P, Traupe H. Hereditary angioedema with normal C1-inhibitor activity in women. Lancet. Jul 15 2000;356(9225):213-7. [Medline].

  5. Nielsen EW, Gran JT, Straume B, et al. Hereditary angio-oedema: new clinical observations and autoimmune screening, complement and kallikrein-kinin analyses. J Intern Med. Feb 1996;239(2):119-30. [Medline].

  6. US Food and Drug Administration (FDA). FDA Approves Berinert to Treat Abdominal Attacks, Facial Swelling Associated With Hereditary Angioedema. FDA Web site. Available at http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm186257.htm. Accessed October 23, 2009.

  7. 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].

  8. Bork K, Witzke G. Long-term prophylaxis with C1-inhibitor (C1 INH) concentrate in patients with recurrent angioedema caused by hereditary and acquired C1-inhibitor deficiency. J Allergy Clin Immunol. Mar 1989;83(3):677-82. [Medline].

  9. Cicardi M, Bergamaschini L, Cugno M, et al. Long-term treatment of hereditary angioedema with attenuated androgens: a survey of a 13-year experience. J Allergy Clin Immunol. Apr 1991;87(4):768-73. [Medline].

  10. Gelfand JA, Sherins RJ, Alling DW, Frank MM. Treatment of hereditary angioedema with danazol. Reversal of clinical and biochemical abnormalities. N Engl J Med. Dec 23 1976;295(26):1444-8. [Medline].

  11. Sheffer AL, Fearon DT, Austen KF. Clinical and biochemical effects of stanozolol therapy for hereditary angioedema. J Allergy Clin Immunol. Sep 1981;68(3):181-7. [Medline].

  12. Gadek JE, Hosea SW, Gelfand JA, et al. Replacement therapy in hereditary angioedema: successful treatment of acute episodes of angioedema with partly purified C1 inhibitor. N Engl J Med. Mar 6 1980;302(10):542-6. [Medline].

  13. Waytes AT, Rosen FS, Frank MM. Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate. N Engl J Med. Jun 20 1996;334(25):1630-4. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

hereditary angioedema, C1 inhibitor deficiency, C1 esterase inhibitor deficiency, HAE, oedema, complement deficiency induced angioedema, angioneurotic edema, acquired angioedema, AAE, complement component 1 inhibitor deficiency, laryngeal edema, Quincke's disease

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Michael M Frank, MD, Distinguished Professor, Departments of Pediatrics and Medicine, Professor, Pediatric Allergy and Immunology Division, Duke University School of Medicine
Michael M Frank, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, and Society for Pediatric Research
Disclosure: Lev Pharma Grant/research funds research; CSL Behring Grant/research funds research; DYAX Consulting fee Review panel membership; Jerini Consulting fee Consulting

Medical Editor

Stephen Rosenfeld, MD, Professor Emeritus, Department of Medicine, Allergy, Immunology and Rheumatology Unit, University of Rochester School of Medicine and Dentistry
Stephen Rosenfeld, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American Federation for Clinical Research, Clinical Immunology Society, and Medical Society of the State of New York
Disclosure: Elan Ownership interest None; Invitrogen Ownership interest None; Merck Ownership interest None; Pfizer Ownership interest None; Medco Health Ownership interest None; Millipore Ownership interest None

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Samuel R Marney, Jr, MD, Director, Associate Professor, Department of Internal Medicine, Division of Allergy and Immunology, Vanderbilt University School of Medicine
Samuel R Marney, Jr, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, and Tennessee Medical Association
Disclosure: Nothing to disclose.

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Michael A Kaliner, MD, Clinical Professor of Medicine, George Washington University School of Medicine; Chief, Section of Allergy and Immunology, Washington Hospital Center; Medical Director, Institute for Asthma and Allergy
Michael A Kaliner, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Clinical Investigation, American Thoracic Society, and Association of American Physicians
Disclosure: Abbott Consulting fee Consulting; Alcon Consulting fee Consulting; Glaxo Consulting fee Consulting; Greer Consulting fee Consulting; Sanofi Consulting fee Consulting; Schering Consulting fee Consulting; Teva  Consulting; Meda Honoraria Speaking and teaching

 
 
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.