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CME: Hereditary Angioedema (HAE): Therapeutic Updates, Best Practices, and Barriers to Care

Activity Description / Statement of Need:

In this online CME self-learning program:


Hereditary angioedema (HAE) is a rare, debilitating, and potentially life-threatening disease due to C1-inhibitor (C1-INH) deficiency with an estimated frequency of 1 in 50,000 people. HAE is characterized by recurrent edema attacks and the cutaneous attacks can be disabling, with the skin, gastrointestinal tract, and upper airways are most commonly affected and with a persistent risk to the patient of acute events of laryngeal swelling that may prove fatal if not treated in a timely manner. Angioedema in general can be confused with cellulitis, Graves disease, blepharochalasis, eosinophilic fasciitis, or amyloidosis which can lead to delays in diagnosis, and inappropriate treatment poses the risk of adverse events, unnecessary surgical interventions, a higher burden of misery, and a potentially higher rate of morbidity and mortality. There are a number of gaps present in the optimal management of patients with HAE. The most authoritative HAE guidelines U.S. guidelines were published in 2013. Communicating related information to healthcare professionals in a timely manner is a demonstrated need.  The literature suggests that practicing healthcare professionals are oftentimes unable to keep up with the steady publishing of literature and evolution of clinical practice; some literature suggests it takes up to five years to fully adopt guidelines into practice once they are published. Recent years have seen advances in therapy, and there are a wide number of treatment options in the acute management of patients, including several C1 esterase inhibitors (some plasma derived, some recombinant, and one approved for prophylactic treatment of HAE), a plasma kallikrein inhibitor, and a bradykinin receptor antagonist.





Introduction, Disclosures

A review primer: epidemiologic, pathophysiologic, etiologic features, and clinical signs and symptoms [Learning Objectives #1 and 2]

  • Common ground: definitions, background, statistics - epidemiology of and risk factors for hereditary angioedema (HAE)
  • Leading abnormalities and causes, as expressed in heritability and de novo mutations
  • Pathophysiology, clinical presentation and clinical pearls, diagnostic practice guidance, and missed opportunities in patients’ presentations

Pharmacotherapeutic management of HAE [Learning Objective #3]

  • Pharmacotherapy detailing: review and updates, acute and prophylactic therapy
  • Best practice: putting it all together
  • Patient case(s)

Barriers to optimal care in patients diagnosed with HAE [Learning Objective #4]

  • Delay in diagnosis
  • The psychosocial, humanistic burden of illness
  • Other factors

Summary, conclusions, and best practice recap

Target Audience:

Healthcare professionals who are: allergists; immunologist; physician assistants; nurse practitioners; pharmacists; or those otherwise treat or clinically encounter patients with HAE.

This program is supported by an educational grant from Shire

Release Date: August 22, 2016 -- Expiration Date: August 22, 2018

Faculty: R. Gentry Wilkerson, MD


Learning Objectives

By the end of the session the participant will be able to:

  • Describe the pathophysiology and challenges preventing the early diagnosis of HAE
  • Apply existing diagnostic recommendations and criteria to practice in the management of HAE
  • Identify the present treatment options currently available for management of HAE and apply them to patient cases using evidence-based medicine
  • Describe barriers to care in HAE and suggest strategies for ameliorating them




Faculty Disclosure and Resolution of COI

As a provider of continuing medical education, it is the policy of ScientiaCME to ensure balance, independence, objectivity, and scientific rigor in all of its educational activities. In accordance with this policy, faculty and educational planners must disclose any significant relationships with commercial interests whose products or devices may be mentioned in faculty presentations, and any relationships with the commercial supporter of the activity. The intent of this disclosure is to provide the intended audience with information on which they can make their own judgments. Additionally, in the event a conflict of interest (COI) does exist, it is the policy of ScientiaCME to ensure that the COI is resolved in order to ensure the integrity of the CME activity. For this CME activity, any COI has been resolved thru content review ScientiaCME.


Faculty Disclosure:  R. Gentry Wilkerson, MD, Assistant Professor; Director of Clinical Research; Assistant Residency Program Director, Department of Emergency Medicine, University of Maryland School of Medicine, Baltimore, MD discloses that he has received grant and/or research support from Redhill Biopharma, Shire, Novartis, and SNBL.


Disclosures of Educational Planners: Charles Turck, PharmD, BCPS, BCCCP is an officer and part owner of ScientiaCME, LLC, and has no relevant conflicts of interest to disclose.


Commercial Support Disclosure: This program is supported by an educational grant from Shire


  • Read the learning objectives above
  • Take the Pre-Test (optional). Completion of the pre-test will help us evaluate the knowedge gained by participating in this CME activity.
  • View the online activity. You may view this is in more than one session, and may pause or repeat any portion of the presentation if you need to.
  • Minimum participation threshold: Take the post-test. A score of 70% or higher is required to pass and proceed to the activity evaluation.
  • Complete the activity evaluation and CME registration. A CE certificate will be emailed to you immediately.

Cultural and Linguistic Competence

System Requirements

Windows 7 or above
Internet Explorer 8
*Adobe Acrobat Reader
Mac OS 10.2.8
Safari or Chrome or Firefox
*Adobe Acrobat Reader
Internet Explorer is not supported on the Macintosh

*Required to view Printable PDF Version

Perform Pre-Test (optional)

Please take a few minutes to participate in the optional pre-test. It will help us measure the knowledge gained by participating in this activity.

Additional Courses That Are Related To This Activity

Primary Immunodeficiency: Updates to Screening & Treatment