When is prophylaxis for hereditary angioedema necessary?

Craig, Timothy J.; Riedl, Marc A.; Dykewicz, Mark S.; Gower, Richard; Baker, James; Edelman, Frank J.; Hurewitz, David; Jacobs, Joshua; Kalfus, Ira

doi:10.1016/s1081-1206(10)60506-6

Cited by 66 publications

(83 citation statements)

References 27 publications

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“…HAE significantly impacts the ability of a patient to work or go to school; these impairments are comparable to those experienced by patients with severe asthma or Crohn's disease[36]. Patients may develop narcotic dependence while trying to appropriately manage frequent and severe abdominal pain[37]. Additional care may be required to manage the psychologic impact of HAE disease[37].…”

Section: Consequencesmentioning

confidence: 99%

“…Patients may develop narcotic dependence while trying to appropriately manage frequent and severe abdominal pain[37]. Additional care may be required to manage the psychologic impact of HAE disease[37]. Patients with HAE are more likely to suffer symptoms of depression than the general population, with 42.5% of patients showing at least mild symptoms of depression[36].…”

Section: Consequencesmentioning

confidence: 99%

“…Long-term, or routine, prophylaxis is an important treatment option for patients with HAE. Patients who can be considered for prophylactic therapy are those who experience frequent or severe attacks, have had a previous laryngeal attack, have significant anxiety and poor quality of life as a result of HAE, have limited access to emergency medical care, or choose to receive prophylaxis[37]. In addition, long-term prophylaxis is indicated in patients for whom acute therapy is ineffective or unavailable[37].…”

Section: Treatment Approachesmentioning

confidence: 99%

“…Patients who can be considered for prophylactic therapy are those who experience frequent or severe attacks, have had a previous laryngeal attack, have significant anxiety and poor quality of life as a result of HAE, have limited access to emergency medical care, or choose to receive prophylaxis[37]. In addition, long-term prophylaxis is indicated in patients for whom acute therapy is ineffective or unavailable[37]. Attenuated (17-alpha alkylated) androgens have long been the gold standard for prophylaxis in numerous countries, with danazol, stanazolol, and oxandrolone being used more frequently than other androgens.…”

Section: Treatment Approachesmentioning

confidence: 99%

“…The antifibrinolytic agents, ε -aminocaproic acid and tranexamic acid, are not approved in the United States but have been used extensively in some European countries for long-term prophylaxis, especially in children and pregnant women in whom androgen therapy is contraindicated because of significant risk of adverse effects[4,8,37]. Antifibrinolytics may also be useful in other patients in whom attenuated androgens or intravenous therapy is not appropriate.…”

Section: Treatment Approachesmentioning

confidence: 99%

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Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Gower¹,

Busse²,

Aygören‐Pürsün³

et al. 2011

World Allergy Organization Journal

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Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease resulting from a mutation in the C1-inhibitor gene. HAE is characterized by recurrent attacks of intense, massive, localized subcutaneous edema involving the extremities, genitalia, face, or trunk, or submucosal edema of upper airway or bowels. These symptoms may be disabling, have a dramatic impact on quality of life, and can be life-threatening when affecting the upper airways. Because the manifestations and severity of HAE are highly variable and unpredictable, patients need individualized care to reduce the burden of HAE on daily life. Although effective therapy for the treatment of HAE attacks has been available in many countries for more than 30 years, until recently, there were no agents approved in the United States to treat HAE acutely. Therefore, prophylactic therapy is an integral part of HAE treatment in the United States and for selected patients worldwide. Routine long-term prophylaxis with either attenuated androgens or C1-esterase inhibitor has been shown to reduce the frequency and severity of HAE attacks. Therapy with attenuated androgens, a mainstay of treatment in the past, has been marked by concern about potential adverse effects. C1-esterase inhibitor works directly on the complement and contact plasma cascades to reduce bradykinin release, which is the primary pathologic mechanism in HAE. Different approaches to long-term prophylactic therapy can be used to successfully manage HAE when tailored to meet the needs of the individual patient.

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Section: Consequencesmentioning

confidence: 99%

Section: Consequencesmentioning

confidence: 99%

Section: Treatment Approachesmentioning

confidence: 99%

Section: Treatment Approachesmentioning

confidence: 99%

Section: Treatment Approachesmentioning

confidence: 99%

See 3 more Smart Citations

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Gower¹,

Busse²,

Aygören‐Pürsün³

et al. 2011

World Allergy Organization Journal

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MundhÖHle, Pharynx

Deitmer¹

2009

Handbuch HNO 2009

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Therapeutic Approaches in Hereditary Angioedema

Antoniu

2011

Clinic Rev Allerg Immunol

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Hereditary angioedema (HAE) is characterized by acute attacks of edema with multiple localizations, the laryngeal angioedema being the most potentially lethal. In HAE, C1-INH impairments cause episodic increase in kallikrein activity leading to attacks of angioedema. Several therapies have recently become available to treat or to prevent HAE attacks, and others are under evaluation for this indication. Plasma-derived C1-INH, bradykinin receptor antagonists (icatibant), kallikrein inhibitors (ecallantide), or recombinant C1-INH is authorized on the market for HAE attack therapy or prophylaxis. Some of these compounds can be used exclusively to treat HAE attacks, whereas others can also be used as prophylactic therapies. Such therapies, although not available worldwide, can improve disease outcome due to their different mechanisms of action.

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When is prophylaxis for hereditary angioedema necessary?

Cited by 66 publications

References 27 publications

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

MundhÖHle, Pharynx

Therapeutic Approaches in Hereditary Angioedema

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