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

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• 作者：Giulia Marsalli^a ; ^{giuliamarsalli.med@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Silvia Nastasio^a ; Marco Sciveres^b ; Pier Luigi Calvo^c ; Ugo Ramenghi^c ; Simona Gatti^d ; Veronica Albano^d ; Sara Lega^e ; ^f ; Alessandro Ventura^e ; ^f ; Giuseppe Maggiore^a
• 关键词：GCH-AHA ; Giant cell hepatitis with autoimmune hemolytic anemia ; IVIg ; Intravenous immunoglobulin ; ULN ; Upper limit of the normal ; ALT ; Alanine aminotransferase ; GGT ; Gamma-glutamiltransferase
• 刊名：Clinics and Research in Hepatology and Gastroenterology
• 出版年：2016
• 出版时间：February 2016
• 年：2016
• 卷：40
• 期：1
• 页码：83-89
• 全文大小：479 K

文摘

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.