Clinical Characteristics of Fulminant-Type Autoimmune Hepatitis
Clinical Characteristics of Fulminant-Type Autoimmune Hepatitis
Background: Although a few adult cases of fulminant-type autoimmune hepatitis have been reported, their clinical features and prognosis have remained uncertain.
Aim:To assess the clinical features and prognosis of patients with fulminant-type autoimmune hepatitis.
Methods; Eleven patients (10%) diagnosed with fulminant-type autoimmune hepatitis in accordance with the 1999 criteria of the International Autoimmune Hepatitis Group were analysed.
Results:All 11 patients were female, with a median age of 53 years. Five patients survived without liver transplantation, one received a liver transplantation, and five died without liver transplantation. Nine patients (82%) survived for 2 weeks or more following diagnosis, without liver transplantation. Except for the patient receiving a liver transplantation, serum total bilirubin levels measured during the clinical course were significantly higher in non-survivors than in survivors, although the accompanying serum alanine aminotransferase levels measured for the two groups were similar. Most significantly, serum total bilirubin levels in non-survivors worsened during days 8–15, while levels in survivors improved during the same period.
Conclusions: The short-term prognosis for patients with fulminant-type autoimmune hepatitis may be good. However, patients whose serum total bilirubin levels worsen during days 8–15 should be considered for liver transplantation.
Autoimmune hepatitis is recognized as a chronic, primarily periportal hepatitis, associated with hypergammaglobulinaemia and circulating autoantibodies, which, in most cases, responds to immunosuppressive treatment. Diagnosis is established by the criteria defined by the International Autoimmune Hepatitis Group, and the exclusion of other causes.
The established diagnostic criteria have frequently been employed as a useful diagnostic tool in clinical practice, although atypical patients who do not exhibit typical features have also been reported. Patients with acute-onset or fulminant-type autoimmune hepatitis correspond to these cases. Recently, a majority of patients with acute-onset autoimmune hepatitis were reported to present with fulminant hepatic failure.
The clinical features and prognosis of patients with fulminant-type autoimmune hepatitis have remained uncertain, and only a few adult cases have been reported, in detail. Fulminant hepatic failure can progress rapidly to coma and death from cerebral oedema and multiple organ failure, and the only therapy of proven benefit is emergency liver transplantation. In the United States, 4% of cases with fulminant hepatic failure are due to autoimmune hepatitis, and the short-term transplantation-free survival rate of these patients is reported to be low.
In this study, we analysed the clinical features and prognosis of patients with fulminant-type autoimmune hepatitis.
Summary and Introduction
Summary
Background: Although a few adult cases of fulminant-type autoimmune hepatitis have been reported, their clinical features and prognosis have remained uncertain.
Aim:To assess the clinical features and prognosis of patients with fulminant-type autoimmune hepatitis.
Methods; Eleven patients (10%) diagnosed with fulminant-type autoimmune hepatitis in accordance with the 1999 criteria of the International Autoimmune Hepatitis Group were analysed.
Results:All 11 patients were female, with a median age of 53 years. Five patients survived without liver transplantation, one received a liver transplantation, and five died without liver transplantation. Nine patients (82%) survived for 2 weeks or more following diagnosis, without liver transplantation. Except for the patient receiving a liver transplantation, serum total bilirubin levels measured during the clinical course were significantly higher in non-survivors than in survivors, although the accompanying serum alanine aminotransferase levels measured for the two groups were similar. Most significantly, serum total bilirubin levels in non-survivors worsened during days 8–15, while levels in survivors improved during the same period.
Conclusions: The short-term prognosis for patients with fulminant-type autoimmune hepatitis may be good. However, patients whose serum total bilirubin levels worsen during days 8–15 should be considered for liver transplantation.
Introduction
Autoimmune hepatitis is recognized as a chronic, primarily periportal hepatitis, associated with hypergammaglobulinaemia and circulating autoantibodies, which, in most cases, responds to immunosuppressive treatment. Diagnosis is established by the criteria defined by the International Autoimmune Hepatitis Group, and the exclusion of other causes.
The established diagnostic criteria have frequently been employed as a useful diagnostic tool in clinical practice, although atypical patients who do not exhibit typical features have also been reported. Patients with acute-onset or fulminant-type autoimmune hepatitis correspond to these cases. Recently, a majority of patients with acute-onset autoimmune hepatitis were reported to present with fulminant hepatic failure.
The clinical features and prognosis of patients with fulminant-type autoimmune hepatitis have remained uncertain, and only a few adult cases have been reported, in detail. Fulminant hepatic failure can progress rapidly to coma and death from cerebral oedema and multiple organ failure, and the only therapy of proven benefit is emergency liver transplantation. In the United States, 4% of cases with fulminant hepatic failure are due to autoimmune hepatitis, and the short-term transplantation-free survival rate of these patients is reported to be low.
In this study, we analysed the clinical features and prognosis of patients with fulminant-type autoimmune hepatitis.
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