Article

Autoimmune Hepatitis: A Review of Current Diagnosis and Treatment

Division of Rheumatology, Department of Medicine, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
Hepatitis research and treatment 05/2011; 2011(2090-1364):390916. DOI: 10.1155/2011/390916
Source: PubMed

ABSTRACT Autoimmune hepatitis (AIH) is a chronic inflammatory disorder characterized by periportal inflammation, elevated immunoglobulins, autoantibodies, and a dramatic response to immunosuppression. An environmental agent is hypothesized to trigger an immune-mediated attack directed against liver antigens in genetically predisposed individuals. A plethora of clinical presentations can be seen ranging from chronic indolent disease to fulminant hepatic failure, and diagnosis requires exclusion of other causes of liver disease. Corticosteroid therapy must be instituted early and modified in an individualized fashion. Treatment decisions are often complicated by the diverse clinical manifestations, uncertainty about natural history, evolving ideas about treatment end points, and a multitude of alternative immunosuppressive agents. Achieving normal liver tests and tissue is the ideal treatment end point, but needs to be weighed against the risk of side effects. Decompensated patients may benefit from early liver transplantation. Long-term prognosis is excellent with early and aggressive initiation of therapy. Our paper discusses AIH, giving a detailed overview of its clinical presentation, risk factors, immunopathogenesis, up-to-date diagnostic criteria, current updates in therapy with a brief discussion of AIH in pregnancy, and long-term implications for cirrhosis and hepatocellular carcinoma in AIH patients.

0 Followers
 · 
186 Views
  • Source
    • "Typical symptoms are nonspecific and can include fatigue, jaundice, nausea, and abdominal pain. It is a relatively rare disease affecting women more than men (3.6 : 1 ration) with mean incidence of 1-2 per 100,000 persons per year and prevalence of 11–17 per 100,000 persons [1] [2]. The diagnosis is based on histological findings and presence of one or more specific autoantibodies (ANA and anti-SMA for type 1 and anti-LKM- 1 for type 2). "
    [Show abstract] [Hide abstract]
    ABSTRACT: Autoimmune hepatitis (AIH) is an inflammatory condition of the liver that has a multitude of clinical presentations from chronic hepatitis to acute fulminant hepatitis. AIH diagnosis is typically suspected after ruling out other causes of hepatitis (such as vial hepatitis, hemochromatosis, Wilson's disease, and primary biliary cirrhosis) through serological tests and by findings of high titers of certain autoantibodies (ANA and anti-SMA for type 1 AIH and anti-LKM-1 for type 2 AIH). AIH like most inflammatory conditions is associated with increased ferritin levels (acute-phase reactant) but typically near-normal transferrin saturation. The presence of excessive ferritin level in absence of high-transferrin saturation helps differentiate secondary iron overload from hemochromatosis where transferrin saturation is typically high. We herein describe a case of AIH that presented with high ferritin levels and transferrin saturation suggesting a diagnosis of hemochromatosis and needed arduous work-up to arrive at accurate diagnosis of AIH.
    08/2013; 2013:872987. DOI:10.1155/2013/872987
  • [Show abstract] [Hide abstract]
    ABSTRACT: Experimental and clinical evidence suggests that cytokeratins (CK), among other physiological functions, are expressed in hepatocytes and can be released in the bloodstream after acute or chronic inflammatory liver injury. Interest in CK in viral and nonviral hepatitis has been rapidly increasing during the last years, especially as they have been proposed as circulating biomarkers of hepatocyte necrosis and apoptosis. In the present review, we sought to summarize and discuss the alterations in circulating CK levels in different form viral and nonviral hepatitis, as well as their potential relation with liver histology. Understanding the mechanisms of hepatitis impact on CK and vice versa is a promising area of research that will positively enhance our understanding of the complexity of acute and chronic inflammatory liver injury.
    Clinica chimica acta; international journal of clinical chemistry 09/2011; 412(23-24):2031-6. DOI:10.1016/j.cca.2011.09.002 · 2.76 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: The understanding of autoimmune hepatitis (AIH) has evolved in the past two decades since diagnostic criteria were developed. Now with long-term experience with well characterized cohorts, strides have been gained in understanding the true epidemiology and natural history of the disease. Therapeutic trials have also added new tools to the armamentarium in managing this challenging disease. AIH has been demonstrated to be a disease of middle-aged women, with a disease course that frequently progresses to cirrhosis, transplant or death. Despite its rare prevalence, AIH is one of the most common indications for transplantation. Diagnosis remains challenging, and the most recently adopted criteria prove very specific but lack sensitivity in the diagnosis of AIH, particularly when presenting atypically. Recently, drug-induced AIH and IgG4-associated AIH have been proposed as distinct clinicopathological entities. Clinical trials for alternate therapeutics have long been needed, and recently two agents, budesonide and mycophenolate mofeteil, show promise in treating AIH. Increasing evidence has mounted to suggest that AIH is a disease that often requires long-term treatment, and frequently progresses to end-stage liver disease. Further research identifying predictors of poor outcome, optimal therapeutic regimens and duration of treatment is much needed.
    Current opinion in rheumatology 11/2011; 24(1):84-9. DOI:10.1097/BOR.0b013e32834de5d9 · 5.07 Impact Factor

Preview (3 Sources)

Download
3 Downloads
Available from