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


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.

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    • "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). "
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    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.
    Full-text · Article · Aug 2013
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    • "Women are affected more frequently than men (3.6 : 1), but the disease is seen in all ethnic groups and ages [41]. The diagnosis of AIH relies upon specific clinical and laboratory criteria and the exclusion of other viral, genetic, and toxic conditions [41, 42]. "
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    ABSTRACT: Sensory neuronopathies (SNs) are a specific subgroup of peripheral nervous system diseases characterized by primary degeneration of dorsal root ganglia and their projections. Multifocal sensory symptoms often associated to ataxia are the classical features of SN. Several different etiologies have been described for SNs, but immune-mediated damage plays a key role in most cases. SN may herald the onset of some systemic autoimmune diseases, which further emphasizes how important the recognition of SN is in clinical practice. We have thus reviewed available clinical, neurophysiological, and therapeutic data on autoimmune disease-related SN, namely, in patients with Sjögren's syndrome, autoimmune hepatitis, and celiac disease.
    Full-text · Article · Jan 2012
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    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.
    No preview · Article · Sep 2011 · Clinica chimica acta; international journal of clinical chemistry
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