Efficacy of intravenous immunoglobulin therapy in giant cell hepatitis with autoimmune hemolytic anemia: A multicenter study

Giulia Marsalli, S. Nastasio, Marco Sciveres, Pier Luigi Calvo, U. Ramenghi, Simona Gatti, Veronica Albano, S. Lega, Alessandro Ventura, Giuseppe Maggiore

Research output: Contribution to journalArticle

Abstract

Background and 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 2 g/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 prothombin 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.

Original languageEnglish
Pages (from-to)83-89
Number of pages7
JournalClinics and Research in Hepatology and Gastroenterology
Volume40
Issue number1
DOIs
Publication statusPublished - Feb 1 2016

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Autoimmune Hemolytic Anemia
Passive Immunization
Intravenous Immunoglobulins
Giant Cells
Hepatitis
Multicenter Studies
Intravenous Infusions
Immunosuppressive Agents
Liver Diseases
Recurrence
Pharmaceutical Preparations
Hemolytic Anemia
Rare Diseases
Prednisone
Transaminases
Therapeutics
Retrospective Studies
Steroids
Safety

ASJC Scopus subject areas

  • Gastroenterology

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Efficacy of intravenous immunoglobulin therapy in giant cell hepatitis with autoimmune hemolytic anemia : A multicenter study. / Marsalli, Giulia; Nastasio, S.; Sciveres, Marco; Calvo, Pier Luigi; Ramenghi, U.; Gatti, Simona; Albano, Veronica; Lega, S.; Ventura, Alessandro; Maggiore, Giuseppe.

In: Clinics and Research in Hepatology and Gastroenterology, Vol. 40, No. 1, 01.02.2016, p. 83-89.

Research output: Contribution to journalArticle

Marsalli, Giulia ; Nastasio, S. ; Sciveres, Marco ; Calvo, Pier Luigi ; Ramenghi, U. ; Gatti, Simona ; Albano, Veronica ; Lega, S. ; Ventura, Alessandro ; Maggiore, Giuseppe. / Efficacy of intravenous immunoglobulin therapy in giant cell hepatitis with autoimmune hemolytic anemia : A multicenter study. In: Clinics and Research in Hepatology and Gastroenterology. 2016 ; Vol. 40, No. 1. pp. 83-89.
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