This continuing medical education self-study monograph, “Changing Paradigms in Hereditary Angioedema: Pathophysiology, Diagnosis and Treatment,” is certified for physicians.
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FACULTY REVIEWERS
Michael M. Frank, MD
Professor of Pediatrics
Professor of Medicine
Duke University
School of Medicine
Durham, North Carolina
Bruce L. Zuraw, MD
Professor of Medicine
University of California, San Diego
School of Medicine
San Diego, California
Marc A. Riedl, MD
Assistant Professor of Medicine
Section Head, Clinical Immunology and Allergy
University of California, Los Angeles
David Geffen School of Medicine
Los Angeles, California
MEDIA: MONOGRAPH
AMA Release Date: June 22, 2009
CME Available Until: June 22, 2010
CONTENTS
- Hereditary Angioedema: Clinical Signs and Symptoms
- The Early History of Hereditary Angioedema Research
- The Next Discovery: Deficiency of C1 Inhibitor
- Pathophysiology and Immunologic Features of Hereditary Angioedema
- Conventional Treatment Options
- New Therapeutic Strategies
- References
- Learning Assessment
- Evaluation Form
TARGET AUDIENCE
This activity has been designed to meet the educational needs of physicians involved in the care of patients with hereditary angioedema.
PURPOSE
This activity is intended to provide physicians with clinical information that will contribute to improving competence in the diagnosis and treatment of patients with hereditary angioedema.
EDUCATIONAL OBJECTIVES
After completing this activity, the participant should be better able to:
- Describe the pathophysiology and immunologic features of hereditary angioedema (HAE)
- Review the signs and symptoms of patients who present with HAE
- Identify diagnostic considerations in HAE
- Outline therapeutic strategies for prophylaxis and acute HAE attacks
STATEMENT OF NEED
Hereditary angioedema (HAE) is an autosomal dominant disease caused by a functional or quantitative defect in C1 inhibitor. In the United States, HAE is estimated to afflict 4000 to 10,000 people.1 C1inhibitor is a member of the serpin family of serine protease inhibitors and is the major inhibitor of several complement proteases and contact-system proteases.2 During HAE attacks, these signaling cascades are set in motion, resulting in the production of several vasoactive substances, including bradykinin. Patients with HAE suffer from recurrent attacks of intense localized edema involving the skin, airways, and visceral organs. Left untreated, most patients will suffer at least one acute attack per month, resulting in debilitation for 20 to 100 days per year.3 Chronic therapy with attenuated androgens or plasmin inhibitors has been the mainstay of therapy; however, many new therapies for prophylaxis or acute treatment are on the horizon. Clinical trials are ongoing with a C1 inhibitor purified from plasma, a recombinant C1 inhibitor, a kallikrein inhibitor, and a bradykinin type 2 receptor antagonist. The future of HAE treatment appears promising—offering hope to thousands who suffer from this debilitating disease.
1Frank MM. Urticaria and angioedema. In: Goldman L, Bennett JC, eds. Cecil Textbook of Medicine. 21st ed. Philadelphia: WB Saunders;
2000:1440-1445.
2Zuraw BL. Hereditary angioedema. N Engl J Med. 2008;359:1027-1036.
3Cicardi M, Agostini A. Hereditary angioedema. N Engl J Med. 1996;334:1666-1667.
DISCLOSURE OF CONFLICTS OF INTEREST
Postgraduate Institute for Medicine (PIM) requires instructors, planners, managers, and other individuals who are in a position to control the content of this activity to disclose any real or apparent conflict of interest they may have as related to the content of this activity. All identified conflicts of interest are thoroughly vetted by PIM for fair balance, scientific objectivity of studies mentioned in the materials or used as the basis for content, and appropriateness of patient care recommendations.
PHYSICIAN CONTINUING EDUCATION
ACCREDITATION STATEMENT
This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of Postgraduate Institute for Medicine (PIM) and Robert Michael Educational Institute LLC (RMEI). PIM is accredited by the ACCME to provide continuing medical education for physicians.
CREDIT DESIGNATION
PIM designates this educational activity for a maximum of 1.0 AMA PRA Category 1 Credit™. Physicians should only claim credit commensurate with the extent of their participation in the activity.
A statement of credit will be issued only upon receipt of a completed activity Evaluation form and will be mailed to you within 3 weeks.
STATEMENT OF SUPPORT
This activity is jointly sponsored by Robert Michael Educational Institute LLC and Postgraduate Institute for Medicine, and is supported by an educational grant from ViroPharma Incorporated.
FEE INFORMATION
There is no fee for this educational activity.
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FOR MORE INFORMATION:
Additional educational activities offered by Robert Michael Educational Institute LLC can be found at www.RMEI.com or by calling toll-free to 866-770-RMEI.
United States Hereditary Angioedema Association
This non-profit patient advocacy organization is dedicated to expediting US approval of safer and more effective HAE therapies. In addition, the HAEA provides a wide range of services that include clinical trial placement, physician referrals, education, and individualized patient case management.
For more information, visit www.haea.org.
