Current options for the acute treatment and prophylaxis of hereditary angioedema

2012 ◽  
pp. 18-31
Author(s):  
Laraib Tabba ◽  
Timothy J Craig
Keyword(s):  
Hereditary Angioedema ◽  
Acute Treatment

scholarly journals Review of the Manitoba cohort of patients with hereditary angioedema with normal C1 inhibitor

2019 ◽  
Vol 15 (1) ◽  
Author(s):  
Lundy McKibbin ◽  
Colin Barber ◽  
Chrystyna Kalicinsky ◽  
Richard Warrington
Keyword(s):  
Family History ◽  
Tranexamic Acid ◽  
Hereditary Angioedema ◽  
Acute Treatment ◽  
Chart Review ◽  
Positive Family History ◽  
Retrospective Chart Review ◽  
Factor Xii ◽  
C1 Inhibitor ◽  
And Function

Abstract Background Hereditary angioedema with normal C1 inhibitor (HAE-nC1 INH) is a rare, underappreciated condition characterized by recurrent subcutaneous angioedema. The underlying pathophysiology and diagnostic criteria continues to evolve. There is a significant overlap between HAE-nC1 INH and idiopathic nonhistaminergic angioedema, ultimately this may be found to be the same condition. Characterization of cohorts suspected to have either of these conditions is warranted to help refine diagnosis, pathophysiology, and treatment response. Methods A retrospective chart review of 418 patients diagnosed with angioedema was conducted. The following inclusion criteria was used: lack of response to antihistamines, steroids, and epinephrine; normal C4, C1 inhibitor (C1 INH) level and function; lack of urticaria or pruritus; occurrence without offending drugs; and positive family history. Enzyme immunoassays for C1 INH function were performed at the Mayo Clinic. Charts meeting these criteria were reviewed for frequency and type of episodes as well as use and response to therapies. Results 6 patients met the above criteria. 3 of these completed genetic testing, none were found to have factor XII abnormalities. None had angiopoietin 1 or plasminogen gene sequencing. 5 of 6 patients were successfully treated with C1 INH or tranexamic acid for acute treatment of attacks (4 with C1 INH and 1 with tranexamic acid). 4 patients have used Icatibant with good response (typically under 40 min for near full recovery); of these, 3 required Icatibant as acute treatment after other therapies (C1 inhibitor and tranexamic acid) were ineffective. There were 9 patients who otherwise met criteria, but due to a lack of family history were classified as having idiopathic non-histaminergic angioedema. Conclusions This retrospective chart review found 6 HAE-nC1 INH patients in Manitoba. 1 responded to tranexamic acid and not C1 INH, 4 typically responded to C1 INH, and 1 responded exclusively to Icatibant. All patients—4 total—who used Icatibant responded; of these 4 patients, 3 required Icatibant after other therapies had failed.

Hereditary angioedema: On-demand treatment of angioedema attacks

2020 ◽  
Vol 41 (6) ◽  
pp. S26-S29
Author(s):  
Sandra C. Christiansen ◽  
Bruce L. Zuraw
Keyword(s):  
Vascular Permeability ◽  
Hereditary Angioedema ◽  
Acute Treatment ◽  
Prophylactic Treatment ◽  
Unmet Needs ◽  
Burden Of Illness ◽  
Early Administration ◽  
On Demand ◽  
The Past ◽  
Normal Life

The availability of effective acute treatment for angioedema has been fundamental in reducing the burden of illness for patients with hereditary angioedema (HAE). In building on the foundation of scientific advances that elucidate the pathomechanism(s) of attacks related to vascular permeability, novel targeted on-demand treatments have been developed and approved. These therapies have provided the means to arrest episodes of swelling, which, in the past, had the potential to inexorably lead to morbidity, and even mortality, for patients with HAE. Access to these medications, along with an emphasis on early administration and guidance that all attacks are candidates for treatment, has shifted the management paradigm for HAE. Although unmet needs remain, these acute therapies, coupled with advances in prophylactic treatment, have furthered the goal for all patients with HAE to live a normal life.

Sustained Response Following Acute Treatment Of Hereditary Angioedema Attacks With Recombinant Human C1 Esterase Inhibitor

2014 ◽  
Vol 133 (2) ◽  
pp. AB37 ◽  
Author(s):  
H. Henry Li ◽  
Marc A. Riedl ◽  
Jonathan A. Bernstein ◽  
William R. Lumry ◽  
Avner Reshef ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
Acute Treatment ◽  
Sustained Response ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

Anaphylaxis in Response to C1 Esterase Inhibitor in a Patient with Hereditary Angioedema

2007 ◽  
Vol 19 (4) ◽  
pp. 159-163 ◽  
Author(s):  
Marco Cicardi ◽  
Lorenza Zingale ◽  
Andrea Zanichelli ◽  
Daniela Lambertenghi Deliliers
Keyword(s):  
Hereditary Angioedema ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor
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