Efficacy and Safety of Long-Term Prophylaxis with C1 Inhibitor (C1INH) Concentrate in Patients with Hereditary Angioedema (HAE)

Zuraw, Bruce L.; Busse, Paula J.; White, Martha V.; Jacobs, Joshua; Lumry, William R.; Baker, James; Hurewitz, David; Manning, M. A.; Koleilat, Majed; Friedman, Roger; Wilkinson, Robert H.; Cartwright, William; Tanner, David A.; Kohler, Gary; Gunther, Glenne

doi:10.1016/j.jaci.2007.12.1077

Cited by 4 publications

(5 citation statements)

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“…Twenty-two patients were involved in a 24-week cross-over study receiving C1-INH 1000 U twice weekly for 12 weeks and placebo (normal saline) treatment for 12 weeks. The subjects had fewer attacks during the C1-INH treatment period than the placebo period (mean 6.1 versus 12.7, p Ͻ 0.0001) (Zuraw et al 2008).…”

Section: Resultsmentioning

confidence: 90%

Treatment of hereditary angioedema with plasma-derived C1 inhibitor

Prematta

Craig³

2008

TCRM

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Section: Resultsmentioning

confidence: 90%

Treatment of hereditary angioedema with plasma-derived C1 inhibitor

Prematta

Craig³

2008

TCRM

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“…Subjects received C1-INH 1000 U twice weekly or placebo for 12 weeks and then randomized to the other arm. Results demonstrated that subjects had fewer attacks during the C1-INH treatment period than the placebo period ( P < 0.0001) 23. While on chronic replacement, as compared to when subjects were on placebo, subjects not only had fewer attacks, but when they had attacks, the attacks were less severe and shorter in duration.…”

Section: History Of C1-inh Use For Prophylaxis In Haementioning

confidence: 92%

“…In a previous study by Zuraw et al, 23 22 patients were investigated over a 24-week period. Subjects received C1-INH 1000 U twice weekly or placebo for 12 weeks and then randomized to the other arm.…”

Section: History Of C1-inh Use For Prophylaxis In Haementioning

confidence: 99%

“…Results demonstrated that subjects had fewer attacks during the C1-INH treatment period than the placebo period ( P < 0.0001). 23 While on chronic replacement, as compared to when subjects were on placebo, subjects not only had fewer attacks, but when they had attacks, the attacks were less severe and shorter in duration. Of the 22 patients, 2 worsened while on chronic replacement therapy and one had no change in attacks.…”

Section: History Of C1-inh Use For Prophylaxis In Haementioning

confidence: 99%

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Cinryze™ as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety

Lunn

Santos²,

Craig³

2010

JBM

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Hereditary angioedema (HAE) is a clinical disorder characterized by a deficiency of C1 esterase inhibitor (C1-INH). HAE has traditionally been divided into two subtypes. Unique among the inherited deficiencies of the complement system, HAE Types I and II are inherited as an autosomal dominant disorder. The generation of an HAE attack is caused by the depletion and/or consumption of C1-inhibitor manifested as subcutaneous or submucosal edema of the upper airway, face, extremities, or gastrointestinal tract. Attacks can be severe and potentially life-threatening, particularly with laryngeal involvement. Despite the availability of C1-INH for the treatment of HAE since the 1980s in Europe and other countries, HAE treatment in the United States was limited to androgen therapy. The human plasma-derived C1 esterase inhibitor (Cinryze™), distributed by Lev Pharmaceuticals, was approved in October 2008 for the prevention of HAE attacks based on the results of a phase III clinical trial. This review aims to describe the history of C1-INH replacement in HAE as well as the pharmacology, efficacy and safety of C1-INH, concentrating on Cinryze as the first approved chronic replacement treatment for the prophylaxis of HAE attacks.

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“…C1-INH is purified from the pooled plasma of volunteer donors and is subject to one or more additional treatments to minimize risk of pathogen transmission. Current C1-INH concentrates have proven safe and effective in double-blind placebo-controlled trials9,13–18 and in clinical practice14,19–22 C1-INH is also effective in prophylaxis 23–25. Availability has been limited by licensing status, expense, its human source and requirement for intravenous administration, and until recently it has not been available at all in several countries, including the US.…”

Section: Hereditary Angioedema: Clinical Features and Current Managemmentioning

confidence: 99%

Management of acute attacks of hereditary angioedema: potential role of icatibant

Longhurst¹

2010

VHRM

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Icatibant (Firazyr®) is a novel subcutaneous treatment recently licensed in the European Union for acute hereditary angioedema. Hereditary angioedema, resulting from inherited partial C1 inhibitor deficiency, is a disabling condition characterized by intermittent episodes of bradykinin-mediated angioedema. Icatibant blocks bradykinin B2 receptors, attenutating the episode. Randomized double-blind, placebo-controlled trials of icatibant, showed significant superiority over oral tranexamic acid in 74 European patients and a trend to improvement in a similar US trial comparing icatibant with placebo in 55 patients. Outcomes for several endpoints did not reach significance in the US trial, perhaps because of low participant numbers and confounding factors: a further trial is planned. Open label studies have shown benefit in multiple treatments for attacks at all sites. Approximately 10% of patients require a second dose for re-emergent symptoms, usually 10 to 27 hours after the initial treatment. Its subcutaneous route of administration, good tolerability and novel mode of action make icatibant a promising addition to the limited repertoire of treatments for hereditary angioedema.

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Efficacy and Safety of Long-Term Prophylaxis with C1 Inhibitor (C1INH) Concentrate in Patients with Hereditary Angioedema (HAE)

Cited by 4 publications

References 0 publications

Treatment of hereditary angioedema with plasma-derived C1 inhibitor

Treatment of hereditary angioedema with plasma-derived C1 inhibitor

Cinryze™ as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety

Management of acute attacks of hereditary angioedema: potential role of icatibant

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Efficacy and Safety of Long-Term Prophylaxis with C1 Inhibitor (C1INH) Concentrate in Patients with Hereditary Angioedema (HAE)

Cited by 4 publications

References 0 publications

Treatment of hereditary angioedema with plasma-derived C1 inhibitor

Treatment of hereditary angioedema with plasma-derived C1 inhibitor

Cinryze&trade; as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety

Management of acute attacks of hereditary angioedema: potential role of icatibant

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Product

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Cinryze™ as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety