Treatment of hereditary angioedema: Current perspectives

Svetlana I. Krassilnikova, Yuriy S. Nikiforov, Timothy Craig

Research output: Contribution to journalReview article

6 Citations (Scopus)

Abstract

Hereditary angioedema (HAE) is a rare familial disease characterized by recurrent self-limiting episodes of soft tissue swelling affecting different parts of the body. Acute HAE attacks range from benign, but disfiguring skin edema, to painful abdominal, and even life-threatening laryngeal attacks. The disease is caused by an aberrant C1 esterase inhibitor (C1-INH), which regulates complement, fibrinolytic, and contact pathways. Elevated serum level of bradykinin as a result of contact pathway activation is thought to be the major mediator of pain and edema formation in HAE. Current therapy of acute HAE attacks is limited and mainly offers symptom control. In the United States only fresh frozen plasma provides some reconstitution of C1-INH, but the efficacy and safety of this treatment is controversial. In some European countries two human derived C1-INH concentrates have been used successfully. Prophylactic therapy for patients with frequent HAE attacks is confined to attenuated androgens and in some countries anti-fibrinolytics and C1-INH are also used. To satisfy the unmet needs, investigation of one recombinant C1-INH, two drugs working on bradykinin pathway and two human derived C1-INH concentrates are underway. This review article also discusses some recent patents related to the filed.

Original languageEnglish (US)
Pages (from-to)166-174
Number of pages9
JournalRecent Patents on Inflammation and Allergy Drug Discovery
Volume2
Issue number3
DOIs
StatePublished - Jan 1 2008

Fingerprint

Complement C1 Inhibitor Protein
Hereditary Angioedemas
Bradykinin
Edema
Therapeutics
Patents
Rare Diseases
Human Body
Androgens
Safety
Pain
Skin
Serum
Pharmaceutical Preparations

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Drug Discovery

Cite this

Krassilnikova, Svetlana I. ; Nikiforov, Yuriy S. ; Craig, Timothy. / Treatment of hereditary angioedema : Current perspectives. In: Recent Patents on Inflammation and Allergy Drug Discovery. 2008 ; Vol. 2, No. 3. pp. 166-174.
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Treatment of hereditary angioedema : Current perspectives. / Krassilnikova, Svetlana I.; Nikiforov, Yuriy S.; Craig, Timothy.

In: Recent Patents on Inflammation and Allergy Drug Discovery, Vol. 2, No. 3, 01.01.2008, p. 166-174.

Research output: Contribution to journalReview article

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