Management of acute attacks of hereditary angioedema: role of ecallantide

Figures & data

Table 1 Diagnosis of types 1, 2, 3, and idiopathic HAE

Laboratory test	Type 1 HAE	Type 2 HAE	Type 3 and idiopathic HAE
C4 concentration	Low	Low	Normal
C1-INH concentration	Low	Normal/high	Normal
C1-INH function	Low	Low	Normal

Abbreviations: C1-INH, C1-inhibitor; HAE, hereditary angioedema.

Figure 1 The pathophysiology of bradykinin formation via the contact pathway.

Notes: Functioning C1-INH inhibits the formation of bradykinin. ACE inhibitors impair the degradation of bradykinin.
Abbreviations: ACE, angiotensin-converting enzyme; C1-INH, C1-inhibitor; fXII, factor XII; HMWK, high-molecular-weight kininogen.

Figure 2 Mechanisms of action of the drugs used for treatment of acute HAE attacks.

Abbreviations: C1-INH, C1-inhibitor; fXII, factor XII; HAE, hereditary angioedema; HMWK, high-molecular-weight kininogen.

Table 2 Adverse reactions occurring at ≥3% than placebo in two placebo-controlled clinical trials in patients with HAE treated with ecallantide

Adverse reactions	Ecallantide (N=100), n (%)*	Placebo (N=100), n (%)
Headache	8 (8%)	6 (7%)
Nausea	5 (5%)	1 (1%)
Diarrhea	4 (4%)	3 (4%)
Pyrexia	4 (4%)	0
Injection site reaction	3 (3%)	1 (1%)
Nasopharyngitis	3 (3%)	0

Notes:

* Patients experiencing the same adverse reaction more than one time were only counted once in that category. Adverse reactions of ecallantide compared to placebo from prescribing information package insert. Data from US FDA.²⁶

Abbreviation: HAE, hereditary angioedema.

USFDACenter for Drug Evaluation and Research Application 125277: Ecallantide Summary Review of Regulatory Action [online]2009 Available from: http://www.accessdata.fda.gov.ezproxy.lib.utah.edu/drugsatfda_docs/nda/2009/125277s000SumR.pdfAccessed October 16, 2014

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