Giant cell hepatitis with <scp>C</scp>oombs‐positive haemolytic anaemia: steroid sparing with high‐dose intravenous immunoglobulin and cyclosporine

Lega, Sara; Maschio, Massimo; Taddio, Andrea; Maggiore, Giuseppe; Ventura, Alessandro

doi:10.1111/apa.12114

Cited by 11 publications

(5 citation statements)

References 13 publications

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“…Sequential infusions of IVIg at monthly intervals were found to be associated with a steroid-sparing effect allowing for complete or partial remission in all patients, confirming previous reports in single cases [16,17]. However, the efficacy of periodical infusion of IVIg was temporary and relapse of the hemolytic anemia and/or of liver disease occurred in all patients.…”

Section: Discussionsupporting

confidence: 84%

See 1 more Smart Citation

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

Marsalli

Nastasio

Sciveres

et al. 2016

Clinics and Research in Hepatology and Gastroenterology

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Section: Discussionsupporting

confidence: 84%

“…Results, however, were not conclusive and usually limited to a slight reduction of aminotransferase activity. Repeated IVIg infusions at immunomodulatory doses were reported to induce remission of the liver disease and to allow corticosteroid discontinuation in two infants with GCH-AHA with severe steroid-related side effects [16,17] (Table 1).…”

mentioning

confidence: 99%

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

Marsalli

Nastasio

Sciveres

et al. 2016

Clinics and Research in Hepatology and Gastroenterology

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“…In this patient, a subsequent GCs reduction has been successfully performed through maintaining repeated infusion of IVIg. 41…”

Section: Introductionmentioning

confidence: 99%

Intravenous immunoglobulin: a biological corticosteroid-sparing agent in some autoimmune conditions

Watad

Amital

Shoenfeld

2017

Lupus

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Intravenous immunoglobulin (IVIg) is increasingly used for the treatment of autoimmune and systemic inflammatory diseases. This compound is effective in a wide range of clinical conditions other than primary immunodeficiency, including autoimmune diseases, inflammatory disorders, infections, organ transplantation, and possibly supportive therapy for cancer. Systemic corticosteroids remain the gold standard treatment for many autoimmune diseases, but their long-term use is associated with complications in diverse organs and systems. Osteoporosis, osteonecrosis, cardiovascular disease, infections, and cancer have been associated with this treatment. Therefore, physicians are occasionally forced to withdraw the treatment with steroids. Biological agents may represent a good alternative, but in addition to being very expensive, these agents may have serious side effects. This review aimed to cover the major advances in the use of IVIg as a steroid-sparing agent in some relevant autoimmune diseases.

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“…In most patients there is an initial response to first line therapy, but relapses occur commonly, and second line therapy is required. Several immunosuppressive drugs, commonly used in the treatment of JAIH, have been tried as second line therapy for GCH-AHA, such as cyclosporine (2,7,(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19), cyclophosphamide (2,11), mycophenolate mofetil (10,12,14,15,20,21), 6-mercaptopurine (14,22), tacrolimus (10,15,16,20) and sirolimus (15,16,23), not always successfully (Table 2).…”

Section: Existing and Emerging Therapiesmentioning

confidence: 99%

“…In case of severe liver dysfunction and/or severe anemia, transitory remission has been achieved with intravenous immunoglobulins (IVIg) (2,(9)(10)(11)(13)(14)(15)(17)(18)(19)(20)(21)(22)(23)(26)(27)(28)(29). IVIg has been used mainly at replacement dose and in single administration, for treating AHA before the onset of liver disease or in case of hepatitis relapses.…”

Section: Existing and Emerging Therapiesmentioning

confidence: 99%

Giant cell hepatitis associated with autoimmune hemolytic anemia: an update

Nastasio

Matarazzo²,

Sciveres

et al. 2021

Transl Gastroenterol Hepatol

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Giant cell hepatitis associated with autoimmune hemolytic anemia (GCH-AHA) is a rare and severe disease characterized by autoimmune hemolysis associated with acute liver injury, histologically defined by widespread giant cell transformation. It occurs after the neonatal period, most commonly in the first year of life and uniquely affects pediatric patients. It is still poorly understood and likely underdiagnosed, although in recent years there have been advances in the understanding of its pathogenesis and the liver injury is now hypothesized to be secondary to a humoral immune mechanism. Although no laboratory test specific for the diagnosis currently exists, given its severity, it is fundamental to rule out GCH-AHA when evaluating a patient in the first year of life presenting with AHA and/or with acute liver disease of unknown etiology. While GCH-AHA is progressive in nature as other autoimmune liver disorders, it differs significantly from juvenile autoimmune hepatitis (JAIH) in that a cure can be achieved after several years of intensive treatment in a portion of patients. Conventional first line therapy consist of prednisone/ prednisolone combined with azathioprine, however, several immunosuppressive drugs, commonly used in the treatment of JAIH have been tried as second line therapy, including cyclosporine, cyclophosphamide, mycophenolate mofetil, 6-mercaptopurine, calcineurin inhibitors, and sirolimus. Intravenous immunoglobulins have also been used in cases of severe liver dysfunction and/or severe anemia allowing for transitory remission. More recently treatment with B-cell depletion has been attempted in some patients and encouraging results have been reported in refractory cases. Although what constitutes optimal treatment has yet to be determined, the recent progress in the understanding of the pathogenetic mechanisms of GCH-AHA have made positive strides, cautiously pointing toward a hopeful prognosis for some of these patients.

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Giant cell hepatitis with Coombs‐positive haemolytic anaemia: steroid sparing with high‐dose intravenous immunoglobulin and cyclosporine

Abstract: N/

Cited by 11 publications

References 13 publications

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

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

Intravenous immunoglobulin: a biological corticosteroid-sparing agent in some autoimmune conditions

Giant cell hepatitis associated with autoimmune hemolytic anemia: an update

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