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.


Thu, 2015-04-30

Pharming Reports On Financial Results For Q1 2015

Wed, 2015-04-29

Dyax Corp. Announces First Quarter 2015 Financial Results

Mon, 2015-04-27

New and Convenient Vial Size of Berinert Accepted by Several European Health Authorities

Tue, 2015-03-31

Dyax Announces Positive Results from Phase 1b Clinical Trial of DX-2930

Fri, 2015-03-27

New hae day :-) website

Thu, 2015-03-19

Nominations to Pharming's Board Of Supervisory Directors

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Thu, 2015-05-28 13:00 - Sun, 2015-05-31 14:00

9th C1 Inhibitor Deficiency Workshop

Venue: Danubius Thermal Hotel Margitsziget, Margitsziget, 1133 Budapest, Hungary
Language: English

It is our great pleasure to invite you to the 9th C1-INH Deficiency Workshop. This event has a history of many years, as well as its nature and objectives are familiar to many of us. Nevertheless, given the continued interest from first-time attendees, we would like to introduce the Workshop for them in brief, as follows.

This conference focuses on bradykinin-mediated angioedemas, and particularly on the types resulting from C1-inhibitor deficiency. The topics of this four-day long event cover a wide range of subjects.

Read more about the workshop here.

Fri, 2015-02-20 10:00 - Tue, 2015-02-24 16:00

AAAAI - Annual Meeting 2015

Venue: Houston, TX, United States of America
Language: English

Mark your calendars and head to Houston, Texas for the premier event in allergy/immunology. You will be able to earn CME/CE, learn about cutting-edge research and enrich your networking connections at the 2015 AAAAI Annual Meeting, February 20-24.

For more information please visit: AAAAI 2015

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Be part of the HAEi Community

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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See the video from the 2012 HAE Global Conference in Copenhagen

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