title: Treatment response after repeated administration of C1 esterase inhibitor for successive acute hereditary angioedema attacks.
abstract: Placebo-controlled studies established the efficacy of replacement therapy with C1 esterase inhibitor (C1-INH) concentrate for treating single acute hereditary angioedema (HAE) attacks, but only limited data from prospective studies are available on repeated treatment of successive HAE attacks. This study evaluates the association between repeated treatments with 20 U/kg of C1-INH concentrate (Berinert; CSL Behring, Marburg, Germany) for HAE attacks at any body location and treatment response. In a post hoc analysis of an open-label extension study (International Multicenter Prospective Angioedema C1-INH Trial [I.M.P.A.C.T.2]), the association between repeated treatment with C1-INH and times to onset of symptom relief and complete resolution of HAE symptoms was assessed in patients who were treated for at least 15 attacks by linear regression on the ordinal attack number. Eighteen patients received C1-INH concentrate for at least 15 HAE attacks over a mean duration of 34 months. Demographic and baseline characteristics of these patients were similar to those of all patients in the study. The distribution of body locations and the intensity of HAE attacks were similar for each of the first 15 attacks and subsequent attacks. The extent of previous use of C1-INH concentrate had no effect on the time to onset of symptom relief, the time to complete resolution of HAE symptoms, or the time between attacks treated with C1-INH concentrate; the median of individual linear regression coefficients was not statistically significantly different from 0. Treatment with 20 U/kg of C1-INH concentrate provided consistent treatment response in patients treated for multiple successive HAE attacks at any body location. (Clinicaltrials.gov identifier: NCT00292981).
authors.affiliation: Penn State University, Hershey, Pennsylvania 17033, USA. tcraig@hmc.psu.edu
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Craig
authors.forename: Againdra K
authors.initials: AK
authors.lastname: Bewtra
authors.forename: David
authors.initials: D
authors.lastname: Hurewitz
authors.forename: Robyn
authors.initials: R
authors.lastname: Levy
authors.forename: Gerti
authors.initials: G
authors.lastname: Janss
authors.forename: Kraig W
authors.initials: KW
authors.lastname: Jacobson
authors.forename: Flint
authors.initials: F
authors.lastname: Packer
authors.forename: Jonathan A
authors.initials: JA
authors.lastname: Bernstein
authors.forename: Mikhail A
authors.initials: MA
authors.lastname: Rojavin
authors.forename: Thomas
authors.initials: T
authors.lastname: Machnig
authors.forename: Heinz-Otto
authors.initials: HO
authors.lastname: Keinecke
authors.forename: Richard L
authors.initials: RL
authors.lastname: Wasserman
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D054179:Angioedemas, Hereditary / Q000188:drug therapy* / Q000453:epidemiology*; D002648:Child; D050718:Complement C1 Inhibitor Protein / Q000008:administration & dosage* / Q000009:adverse effects* / Q000627:therapeutic use; D051056:Complement Inactivating Agents / Q000008:administration & dosage* / Q000009:adverse effects* / Q000627:therapeutic use; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D050718:Complement C1 Inhibitor Protein; D051056:Complement Inactivating Agents