Giant cell hepatitis (GCH) with Coombs-positive autoimmune haemolytic anaemia (AIHA) is a rare clinico pathological entity with early age onset and severe clinical course for which an autoimmune origin has been hypothesized.Aggressive immunosuppressive treatment may control dis-ease progression but should be carried on for at least5 years as relapse is frequent after early discontinuation and carries a poor prognosis. Different immunosuppressive drugs have been tried with varying degrees of benefit and several side effects, but due to few reported cases, nounivocal data exist on which therapy might be the most effective. We describe a patient with GCH with AIHA who initially responded to the association of prednisone,cyclosporine and intravenous immunoglobulin (IVIg) but developed severe steroid side effects. Early tapering of steroid was successfully managed with the association of cyclosporine and monthly infusion of high-dose IVIg
Giant cell hepatitis with Cooms-positive haemolytic anemia: steroid sparing with high dose intravenous immunoglobulin and cyclosporine
MAGGIORE, Giuseppe;
2013
Abstract
Giant cell hepatitis (GCH) with Coombs-positive autoimmune haemolytic anaemia (AIHA) is a rare clinico pathological entity with early age onset and severe clinical course for which an autoimmune origin has been hypothesized.Aggressive immunosuppressive treatment may control dis-ease progression but should be carried on for at least5 years as relapse is frequent after early discontinuation and carries a poor prognosis. Different immunosuppressive drugs have been tried with varying degrees of benefit and several side effects, but due to few reported cases, nounivocal data exist on which therapy might be the most effective. We describe a patient with GCH with AIHA who initially responded to the association of prednisone,cyclosporine and intravenous immunoglobulin (IVIg) but developed severe steroid side effects. Early tapering of steroid was successfully managed with the association of cyclosporine and monthly infusion of high-dose IVIgI documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
