title: Use of recombinant C1 inhibitor in patients with resistant or frequent attacks of hereditary or acquired angioedema.
abstract: BACKGROUND
  Conestat alfa (Ruconest, rhC1INH) is the first recombinant human C1 inhibitor protein (C1INH) for the treatment of acute attacks of hereditary angioedema (HAE).
  
  
  OBJECTIVE
  To assess clinical experience of the first 11 adult patients who received rhC1INH in clinical practice in the UK.
  
  
  METHODS
  Eleven patients (nine HAE type 1, one HAE type 2 and one acquired angioedema with C1 inhibitor deficiency) received between one and six, mostly self-administered, doses of rhC1INH for acute HAE attacks. They were asked to record their time to first response and complete resolution following the treatment. This cohort included our most severely affected and difficult to treat patients.
  
  
  RESULTS
  In most cases, time to first improvement following rhC1INH and complete resolution was recorded as comparable to their typical response to pdC1INH, although 4/11 patients reported that the time to first improvement was much quicker than their average pdC1INH response. Five of the 11 patients continued with rhC1INH as their preferred rescue treatment. Of those who chose not to continue the treatment, four reported a recurrence or early return of symptoms with rhC1INH.
  
  
  CONCLUSIONS
  In our experience, rhC1INH is a beneficial treatment for patients with preference for a C1INH that is not plasma derived and it is suitable for home treatment. In some cases it demonstrates cost saving, especially for heavier patients who require higher doses. In some patients rhC1INH may result in faster resolution of symptoms. It may be associated with an early return of symptoms in patients with exceptionally frequent attacks.
authors.affiliation: Department of Immunology, Barts Health NHS Trust, London E1 2ES, UK.
authors.forename: Ania L
authors.initials: AL
authors.lastname: Manson
authors.affiliation: Department of Immunology, Barts Health NHS Trust, London E1 2ES, UK.
authors.forename: John
authors.initials: J
authors.lastname: Dempster
authors.affiliation: Department of Immunology, Barts Health NHS Trust, London E1 2ES, UK.
authors.forename: Sofia
authors.initials: S
authors.lastname: Grigoriadou
authors.affiliation: Department of Immunology, Barts Health NHS Trust, London E1 2ES, UK.
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Buckland
authors.affiliation: Department of Immunology, Barts Health NHS Trust, London E1 2ES, UK.
authors.forename: Hilary J
authors.initials: HJ
authors.lastname: Longhurst
mesh_terms: D000328:Adult; D000799:Angioedema / Q000188:drug therapy*; D054179:Angioedemas, Hereditary / Q000188:drug therapy*; D003174:Complement C1 Inactivator Proteins / Q000627:therapeutic use*; D050718:Complement C1 Inhibitor Protein; D004351:Drug Resistance; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins / Q000627:therapeutic use; D012008:Recurrence; D055815:Young Adult
chemical_list: D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D011994:Recombinant Proteins; C469952:SERPING1 protein, human
keywords: Recombinant C1 inhibitor; acquired angioedema; hereditary angioedema