title: Efficacy of intravenous immunoglobulin therapy in giant cell hepatitis with autoimmune hemolytic anemia: A multicenter study.
abstract: OBJECTIVE
  Giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA) is a rare disease of infancy, of possible autoimmune mechanism with poor prognosis due to its scarce response to immunosuppressive drugs. The aim of this retrospective multicenter study was to evaluate the efficacy and safety of intravenous immunoglobulin (IVIg) treatment in inducing and maintaining remission of the liver disease, in patients with GCH-AHA.
  
  
  METHODS
  Seven children with GCH-AHA, four newly diagnosed, and three in relapse, being treated with different therapies, received one to three IVIg infusions (0.5 to 2g/kg) in association with other immunosuppressive drugs. Subsequently five of them received monthly sequential IVIg infusions (mean 13.4, range 7-24).
  
  
  RESULTS
  IVIg infusions as first-line therapy associated with prednisone and other immunosuppressive drugs significantly (P=0.04) reduced the aminotransferase activity in all patients and normalized prothrombin activity in the only patient with severe liver dysfunction. Sequential monthly IVIg infusions determined a steroid-sparing effect and allowed a complete or partial remission in all patients, although with temporary efficacy, since relapse of the hemolytic anemia and/or of liver disease occurred in all patients. IVIg infusions were associated with mild side effects in two patients.
  
  
  CONCLUSIONS
  IVIg infusion can be safely and effectively administered in patients with severe GCH-AHA at diagnosis, or in case of relapse, in association with other immunosuppressive drugs. Repeated IVIg infusions may help maintain remission, however, due to their temporary efficacy, they should not be routinely employed.
authors.affiliation: Department of Clinical and Experimental Medicine, University of Pisa, Pediatric Gastroenterology Unit; University Hospital Santa Chiara, Pisa, Italy. Electronic address: giuliamarsalli.med@gmail.com.
authors.forename: Giulia
authors.initials: G
authors.lastname: Marsalli
authors.affiliation: Department of Clinical and Experimental Medicine, University of Pisa, Pediatric Gastroenterology Unit; University Hospital Santa Chiara, Pisa, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Nastasio
authors.affiliation: Paediatric Hepatology and Liver Transplant Unit, UPMC-IsMett, Palermo, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Sciveres
authors.affiliation: Department of Paediatric and Public Health Sciences, University of Torino, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Calvo
authors.affiliation: Department of Paediatric and Public Health Sciences, University of Torino, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
authors.forename: Ugo
authors.initials: U
authors.lastname: Ramenghi
authors.affiliation: Department of Paediatrics, Università Politecnica delle Marche, Ancona, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Gatti
authors.affiliation: Department of Paediatrics, Università Politecnica delle Marche, Ancona, Italy.
authors.forename: Veronica
authors.initials: V
authors.lastname: Albano
authors.affiliation: Institute for Maternal and Child Health, University of Trieste, Trieste, Italy; IRCCS Burlo Garofolo, Trieste, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Lega
authors.affiliation: Institute for Maternal and Child Health, University of Trieste, Trieste, Italy; IRCCS Burlo Garofolo, Trieste, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Ventura
authors.affiliation: Department of Clinical and Experimental Medicine, University of Pisa, Pediatric Gastroenterology Unit; University Hospital Santa Chiara, Pisa, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Maggiore
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications*; D005260:Female; D006505:Hepatitis / Q000150:complications* / Q000188:drug therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D012074:Remission Induction; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous