Autoimmune hepatitis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]
Overview
Autoimmune hepatitis (AH) was first described in the 1950s and has had a variety of names including chronic active hepatitis, lupoid hepatitis, plasma cell hepatitis and autoimmune chronic active hepatitis. It is characterized by circulating autoantibodies and a high serum globulin level. It is important to distinguish AH from other forms of liver disease, as a large percentage of patients respond to therapy and therapy has been shown to delay transplantation, improve the quality of life as well as prolong survival. Lupoid hepatitis (also called Autoimmune hepatitis) is an auto-immune disease which causes liver cirrhosis. It may be associated with systemic lupus erythematosus (SLE) or other connective tissue disorders. 60% of patients have chronic hepatitis that may mimic viral hepatitis, but without serologic evidence of a viral infection. The disease usually affects women and is strongly associated with anti-smooth muscle auto-antibodies. Autoimmune hepatitis is a condition in which the patient's own immune systems attacks the liver causing inflammation and liver cell death. The condition is chronic and progressive. Although the disease is chronic, many patients with autoimmune hepatitis present acutely ill with jaundice, fever and sometimes symptoms of severe hepatic dysfunction, a picture that resembles acute hepatitis. Autoimmune hepatitis usually occurs in women (70 %) between the ages of 15 and 40. Although the term "lupoid" hepatitis was originally used to describe this disease, patients with systemic lupus erythematosus do not have an increased incidence of autoimmune hepatitis and the two diseases are distinct entities. Patients usually present with evidence of moderate to severe hepatitis with elevated serum ALT and AST activities in the setting of normal to marginally elevated alkaline phosphatase and gamma-glutamyltranspeptidase activities. The patient will sometimes present with jaundice, fever, and right upper quadrant pain and occasionally systemic symptoms such as arthralgias, myalgias, polyserositis and thrombocytopenia. Some patients will present with mild liver dysfunction and have only laboratory abnormalities as their initial presentation. Others will present with severe hepatic dysfunction.[1]
Historical Perspective
Autoimmune hepatitis was first to describe under the name of lupoid hepatitis in young females in early 1950. In 1950, Waldenstrom described a form of chronic hepatitis in young women. The term autoimmune hepatitis was first used by the international panel. Gene mutations implicated in the pathogenesis of autoimmune hepatitis is C4 gene.
Classification
According to European Association for the Study of the Liver, There are three types of Autoimmune hepatitis based on the types of antibodies present- AIH type 1, AIH type 2, AIH type 3. Overlap syndrome is a type of syndrome found in a patient who presents with the features of AIH , PBC-AIH or PSC-AIH.
Pathophysiology
Causes
Differentiating Autoimmune Hepatitis from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnosis Criteria
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X Ray
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
References
- ↑ Krawitt, E. L. 1996. Autoimmune hepatitis. New England Journal of Medicine. 334:897-903.