Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency

Levi, M., Choi, G., Picavet, C. & Hack, C. E., Apr-2006, In : Journal of Allergy and Clinical Immunology. 117, 4, p. 904-908 5 p.

Research output: Contribution to journalArticleAcademicpeer-review

  • Marcel Levi
  • Goda Choi
  • Charles Picavet
  • C. Erik Hack

Background: Administration of C1-inhibitor concentrate is effective for prophylaxis and treatment of severe angioedema attacks caused by Cl-inhibitor deficiency. The concentrate should be administered intravenously and hence needs to be administered by health care professionals, which might cause considerable delay in treatment and inconvenience for patients.

Objective: The aim of this study was to investigate the feasibility, efficacy, and safety of on-demand and prophylactic self-administration of C1-inhibitor concentrate in patients with frequent attacks of angioedema.

Methods: Patients with hereditary or acquired Cl-inhibitor deficiency who had very frequent angioedema attacks were trained to self-administer Cl-inhibitor concentrate. The study consisted of 31 patients using on-demand treatment and 12 patients using prophylaxis with C1-inhibitor concentrate. Mean follow-up was 3.5 years.

Results: All patients were capable of self-administering the concentrate, with technical failure rates of self-injection being less than 2%. Times between the onset of the attack and the initiation of relief or complete resolution of symptoms in the on-demand group were significantly shortened (2.2 hours and 7.9 hours, respectively) compared with the situation before the start of self-administration. In the prophylaxis group self-administration of Cl-inhibitor concentrate decreased the angioedema attack rate from 4.0 to 0.3 attacks per month.

Conclusion: Intravenous self-administration of Cl-inhibitor concentrate is a feasible and safe option and results in more rapid and more effective treatment or prevention of severe angioedema attacks in patients with Cl-inhibitor deficiency. Clinical implications: Self-administration of C1-inhibitor concentrate could be a valuable and convenient treatment modality to prevent or treat angioedema attacks in patients with C1-inhibitor deficiency.

Original languageEnglish
Pages (from-to)904-908
Number of pages5
JournalJournal of Allergy and Clinical Immunology
Issue number4
Publication statusPublished - Apr-2006
Externally publishedYes


  • C1-inhibitor deficiency, C1-inhibitor concentrate, hereditary angioedema, acquired angiodema, C1 INHIBITOR CONCENTRATE, EDEMA, DANAZOL, ACTIVATION, EPISODES, THERAPY, ACID

ID: 29062673