Clinicopathological features of severe and fulminant forms of autoimmune hepatitis.
Summary of "Clinicopathological features of severe and fulminant forms of autoimmune hepatitis."
BACKGROUND AND
AIMS:
Diagnosis of the acute presentation of autoimmune hepatitis (AIH) is difficult because patients do not always show typical clinicopathological features of AIH. Although some of them progress to fulminant hepatitis, the survival rate of which is <20% without liver transplantation, their clinicopathological features have remained uncertain. We examined them for a better understanding and improvement of the prognosis of "life-threatening" severe and fulminant AIH.
METHODS:
Clinical, biochemical and pathological features of 28 patients with severe or fulminant AIH and treatment responses were examined retrospectively.
RESULTS:
At the time of admission, mean immunoglobulin G was 2479 +/- 1170 mg/dl, with 7 (25%) patients showing normal levels. Anti-nuclear antibody was =1:40 in 8 (29%). Liver histology showed severe activity in 95% and acute hepatitis in 86% of the patients. Centrilobular necrosis including submassive and massive necrosis was characteristic. Of the 25 patients treated with corticosteroids, 17 responded and 8 did not. Responders to corticosteroids showed younger age and higher prothrombin time (PT) activity than non-responders at the time of corticosteroid administration. The improvement of PT activity during 2 weeks and 4 weeks and total bilirubin level during 4 weeks was statistically significant in responders, but not in non-responders.
CONCLUSIONS:
We should diagnose and treat acute onset AIH patients before they develop into severe and fulminant disease. Performing liver biopsy at the early stage of acute onset AIH, evaluating the biopsy specimens precisely and initiating corticosteroid therapy may be essential for improving the prognosis without liver transplantation.
Affiliation
Department of Medicine and Clinical Oncology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan.
Journal Details
This article was published in the following journal.
Name: Journal of gastroenterology
ISSN: 1435-5922
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20821236
- DOI: http://dx.doi.org/10.1007/s00535-010-0316-3
Medical and Biotech [MESH] Definitions
Liver Failure, Acute
A form of rapid-onset LIVER FAILURE, also known as fulminant hepatic failure, caused by severe liver injury or massive loss of HEPATOCYTES. It is characterized by sudden development of liver dysfunction and JAUNDICE. Acute liver failure may progress to exhibit cerebral dysfunction even HEPATIC COMA depending on the etiology that includes hepatic ISCHEMIA, drug toxicity, malignant infiltration, and viral hepatitis such as post-transfusion HEPATITIS B and HEPATITIS C.
Hepatitis, Viral, Human
INFLAMMATION of the LIVER in humans due to infection by VIRUSES. There are several significant types of human viral hepatitis with infection caused by enteric-transmission (HEPATITIS A; HEPATITIS E) or blood transfusion (HEPATITIS B; HEPATITIS C; and HEPATITIS D).
Hepatitis, Chronic
INFLAMMATION of the LIVER with ongoing hepatocellular injury for 6 months or more, characterized by NECROSIS of HEPATOCYTES and inflammatory cell (LEUKOCYTES) infiltration. Chronic hepatitis can be caused by viruses, medications, autoimmune diseases, and other unknown factors.
Hepadnaviridae
A family of hepatotropic DNA viruses which contains double-stranded DNA genomes and causes hepatitis in humans and animals. There are two genera: AVIHEPADNAVIRUS and ORTHOHEPADNAVIRUS. Hepadnaviruses include HEPATITIS B VIRUS, duck hepatitis B virus (HEPATITIS B VIRUS, DUCK), heron hepatitis B virus, ground squirrel hepatitis virus, and woodchuck hepatitis B virus (HEPATITIS B VIRUS, WOODCHUCK).
Hepatitis A Virus
A species in the genus HEPATOVIRUS containing one serotype and two strains: HUMAN HEPATITIS A VIRUS and Simian hepatitis A virus causing hepatitis in humans (HEPATITIS A) and primates, respectively.
PubMed Articles
Acute and Acute Severe (Fulminant) Autoimmune Hepatitis.
Autoimmune hepatitis frequently has an abrupt onset of symptoms, and it can present with acute liver failure. The abrupt presentation can indicate spontaneous exacerbation of a pre-existent chronic di...
AIM: Cerebral hemodynamic derangement is well known in patients with fulminant hepatic failure. The advent of transcranial Doppler sonography (TCD) enabled noninvasive observation of cerebral hemodyna...
Discrimination of autoimmune hepatitis: autoantibody typing and beyond.
For over 20 years autoimmune hepatitis has been classified into types 1 and 2, an autoantibody-based classification that has stood the test of time. In type 1 autoimmune hepatitis, the main circulati...
Efficacy of Intravenous Glycyrrhizin in the Early Stage of Acute Onset Autoimmune Hepatitis.
BACKGROUND: Acute onset autoimmune hepatitis (AIH) shows acute presentation like acute hepatitis and does not have typical clinicopathological features of AIH. There is no gold standard for making the...
Natural history and treatment of chronic delta hepatitis.
Summary. Chronic delta hepatitis (CDH) represents a severe form of chronic viral hepatitis, induced by the hepatitis delta virus (HDV) in conjunction with the hepatitis B virus (HBV). Delta hepatitis...
Clinical Trials
The purpose of the study is to find out the effects Budesonide, 9 mg daily for one year, has on patients with Primary Biliary Cirrhosis with features of autoimmune hepatitis.
A Randomized Controlled Trial of Lamivudine in Acute Hepatitis B
Since a proportion of patients with Acute Viral Hepatitis-B develop severe hepatitis and fulminant hepatic failure, it is logical to believe that a rapid reduction in the HBV DNA levels by...
The purpose of the study is to determine the role special antibodies play in possibly identifying Autoimmune Hepatitis through the following: Identify the response of specific T cells to...
The MARS® Albumin Dialysis System in Patients With Fulminant and Subfulminant Hepatic Failure
The purpose of this study is to improve the survival rate of those patients with acute fulminant hepatitis through treatment with the MARS® extra-corporal liver-purification system by:...
Role of Cytokines in Hepatitis E Virus Infection During Pregnancy
Hepatitis E virus is a public health problem in several countries of the world where safe drinking water is a problem. HEV is an exclusive cause of epidemic hepatitis in general population...