Post-infantile giant cell hepatitis: the role of steroids

Introduction: Giant cell Hepatitis (GCH) is rare in adults. Various conditions are associated with GCH, however pathogenesis remains unclear. GCH has an ominous prognosis with limited treatment options.

Aims: We report clinical, biochemical, histopathological characteristics and outcome in a group of patients with GCH.

Methods: Six patients with GCH were reviewed (5 male, 1 female, age range 19–65). Four (4/6) patients had features suggestive of autoimmune phenomenon. One (1/6) had chronic hepatitis C infection (HCV) and in one (1/6) underlying condition could not be identified. The follow up period ranged from 2–6 years. Abnormalities in liver blood test were seen in all patients. GCH (post infantile) was confirmed on liver histology.

Results: Significant improvement was seen in four patients after early introduction of steroids. Subsequently these patients were maintained on immunosuppressive therapy. HCV patient became PCR negative after anti-viral therapy, however liver disease progressed to cirrhosis with giant cell formation. Patient without any underlying association for GCH required no treatment and resolved spontaneously.

Conclusions: Our experience demonstrates that early introduction of steroids results in significant clinical and biochemical improvement particularly in patients who had associated autoimmune abnormalities. Sustained immunosupression is highly beneficial in maintaining this response. Follow up indicates that giant cell hepatitis does not always carry an unfavourable prognosis.