Diagnosing HAE is not difficult in principle; however patients frequently get a wrong diagnosis primarily because of its rarity, with many doctors not coming across a single patient with this clinical picture in their entire career. Furthermore, HAE is difficult to diagnose due to its non-specific symptoms, which often resemble those of an allergy, intestinal colic or appendicitis and can lead physicians to a wrong conclusion easily. As a result, it can take a relatively long time for HAE to be diagnosed correctly. However, while the disease remains undetected, the patient could be at risk of suffocation due to acute swelling in the larynx in a severe attack or, in the case of intestinal edema, of undergoing unnecessary endoscopy or even having their appendix removed.(2,12)


Angioedema is a common symptom and most cases of angioedema are due to an allergic reaction. As a nonallergic form of angioedema, symptoms of HAE do not typically present with urticaria and are not caused by the common triggers of an allergic response or do not respond to treatments used in histamine-mediated allergic reactions, i.e. antihistamines, glucocorticoids and epinephrine.

Ultimately, a diagnosis of HAE is confirmed by laboratory tests indicating either a reduced concentration of C1-INH and/or depressed C1-INH activity. A measure of C4 antigen concentration is also important as this parameter is decreased in both HAE types I and II. Assaying C1q concentrations is necessary to differentiate between HAE and acquired angioedema, a non –inherited form of angioedema associated with a C1-INH deficiency due to a chronic and/or serious illness.(2,11,21,25)

Diagnosis is difficult in patients who only have episodes of swelling in the intestine. Initially, further information is obtained by establishing whether anyone else in the patient’s family has had the disease (known as family history). However, only a laboratory analysis can provide final confirmation.


Sun, 2014-10-19

Pharming And Swedish Orphan Biovitrum Amend And Extend Ruconest Distribution Agreement

Tue, 2014-10-07

BioCryst Announces Late Breaker Presentation of OPuS-1 Phase 2 Trial Results at the 23rd EADV Congress

Mon, 2014-09-22

Dyax Corp. Highlights Recent Progress in Licensing and Funded Research Portfolio

Mon, 2014-09-22

Sobi opens the company's new North American office in Massachusetts

Mon, 2014-09-15

Dyax Announces Issuance of Two Key U.S. Patents for DX-2930

Thu, 2014-08-28

Newsletter #4/2014 just released

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Sat, 2014-12-06 09:00 - Tue, 2014-12-09 15:00

WAO International Scientific Conference 2014

Venue: SulAmérica Convention Center, Av Paulo de Frontin, 1 - New City, Centro Rio de Janeiro - RJ, Brazil
Language: English

Advancing the borders of allergy: From treatment to prevention by targeting the environment, infections and the susceptible patient

We are delighted to welcome you to participate in the WAO International Scientific Conference 2014 (WISC 2014) and the XLI Annual Congress of the Brazilian Association of Allergy and Immunology (ASBAI) in Rio de Janeiro, Brazil, 6-9 December 2014.

Thu, 2014-11-06 09:00 - Mon, 2014-11-10 16:00

ACAAI - 2014 Annual Scientific Meeting

Venue: Georgia World Congress Convention Center, Atlanta, GA, United States of America
Language: English

Plan to join your colleagues at the 71st ACAAI Annual Scientific Meeting in Atlanta, Nov. 6-10. Broaden your expertise, enhance your practice management skills and improve patient care.

For more information please visit: ACAAI Annual Meeting 2014

Fri, 2014-10-24 13:00 - Sun, 2014-10-26 12:30

International scientific conference on Expanding Boundaries of our HAE Knowledge

Venue: Bethesda Marriott, 5151 Pools Hill Road, Bethesda, MD 20814, USA
Language: English

This first international scientific meeting on HAE, sponsored by National Institutes of Health in conjunction with the US HAEA Angioedema Center at UCSD, will bring together experts in all types of angioedema, as well as clinical medicine to discuss the emerging basic science.

Read more about the conference here


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HAEi is here to help you. Therefore we have created the HAEi Community that gives you all the bells and whistles you know from other social media (such as Facebook) and even a very sophisticated Forum, where you can discuss with fellow HAE patients and care givers. The only difference is - that here your information stays safe - and will only be shared with other HAE patients and care givers. If you are a HAE patient or directly related to a HAE patient we urge you to register today - and become an important member of our community

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HAEDAYHAEDAYLook out for next hae day :-) on 16 May 2014