Treatment of hereditary angioneurotic oedema (HANE) with tibolone

Clin Endocrinol (Oxf). 2007 Feb;66(2):180-4. doi: 10.1111/j.1365-2265.2006.02704.x.

Abstract

Objective: Eight women, aged 25-58 years, with hereditary angioneurotic oedema (HANE) were treated with tibolone, a synthetic steroid exhibiting oestrogenic, androgenic and progestational activity.

Design: Pilot study.

Results: Tibolone at a dose of 2.5-7.5 mg/day significantly reduced the number and severity of attacks and the number of ampoules of C1-esterase inhibitor (C1-INH) needed for symptomatic therapy. The efficacy of tibolone was comparable to that of danazol, while the androgenic side-effects were considerably reduced.

Conclusions: Tibolone may represent an alternative to danazol administration for the prophylaxis of HANE in women.

Publication types

  • Controlled Clinical Trial

MeSH terms

  • Adult
  • Angioedema / drug therapy*
  • Angioedema / genetics*
  • Complement C1 Inactivator Proteins / therapeutic use
  • Dose-Response Relationship, Drug
  • Drug Administration Schedule
  • Drug Therapy, Combination
  • Female
  • Humans
  • Middle Aged
  • Norpregnenes / therapeutic use*
  • Pilot Projects
  • Treatment Outcome

Substances

  • Complement C1 Inactivator Proteins
  • Norpregnenes
  • tibolone