title: Acquired C1-inhibitor deficiency: 7 patients treated with rituximab.
abstract: BACKGROUND
  Acquired C1-inhibitor deficiency can occur secondary to excessive C1-inhibitor consumption (type I) and be associated with a lymphoid hemopathy, or linked to the presence of anti-C1-inhibitor autoantibodies (type II) in a context of an isolated monoclonal gammopathy, sometimes associated with lymphoproliferation. Efficacy of danazol, tranexamic acid and/or corticosteroids is inconstant. Rituximab efficacy against type II angioedema has been reported.
  
  
  METHODS
  Description of 7 rituximab-treated patients, 6 with type II acquired angioedema and 1 with type I.
  
  
  RESULTS
  Clinical efficacy (only for type II) was complete for 3, partial for 2 and 2 were therapeutic failures. Only 2 patients had improved biological parameters, with normalization of their C1-inhibitor levels and diminished anti-C1-inhibitor autoantibodies, observed 1-9 months after the last infusion of the second rituximab cycle. An associated lymphoproliferation did not affect the response to treatment.
  
  
  CONCLUSIONS
  Rituximab efficacy in the treatment of acquired angioedema is inconstant and might require repeated cycles.
authors.affiliation: Service de Médecine Interne, Hôpital Jean-Verdier (AP-HP), Université Paris 13, avenue du 14 Juillet, 93140, Bondy, France.
authors.forename: Albanne
authors.initials: A
authors.lastname: Branellec
authors.forename: Laurence
authors.initials: L
authors.lastname: Bouillet
authors.forename: Nicolas
authors.initials: N
authors.lastname: Javaud
authors.forename: Arsène
authors.initials: A
authors.lastname: Mekinian
authors.forename: Isabelle
authors.initials: I
authors.lastname: Boccon-Gibod
authors.forename: Claire
authors.initials: C
authors.lastname: Blanchard-Delaunay
authors.forename: Eric
authors.initials: E
authors.lastname: Oksenhendler
authors.forename: Yann
authors.initials: Y
authors.lastname: Ollivier
authors.forename: Bertrand
authors.initials: B
authors.lastname: Dunogué
authors.forename: Stephanie
authors.initials: S
authors.lastname: Amarger
authors.forename: Denise
authors.initials: D
authors.lastname: Ponard
authors.forename: Christian
authors.initials: C
authors.lastname: Drouet
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.forename: Michel
authors.initials: M
authors.lastname: Thomas
authors.forename: Olivier
authors.initials: O
authors.lastname: Fain
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000799:Angioedema / Q000188:drug therapy*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D018951:Antigens, CD20 / Q000276:immunology; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D050718:Complement C1 Inhibitor Protein; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D007155:Immunologic Factors; C469952:SERPING1 protein, human; D000069283:Rituximab