Autoimmune hepatitis

Changed by Jeremy Jones, 20 Sep 2021

Updates to Article Attributes

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Autoimmune hepatitis (AIH) (plural: autoimmune hepatitides) is a rare type of chronic hepatitis, currently classified as "type 1" or "type 2". It may eventually lead to cirrhosis. The role of imaging is primarily to exclude other diagnoses and evaluate for complications.

Epidemiology

It may occur in children or adults, but most patients are affected at 40-60 years old. The incidence in Western Europe is ~0.01-0.02% 2.

There is no predilection for an ethnic group. There is a female predominance: 3.6: 1.

Clinical presentation

Autoimmune hepatitis has a variable clinical presentation. Patients may be asymptomatic or present in acute liver failure (rare), or anywhere between. It tends to have a relapsing-remitting course. There is an association with other autoimmune conditions.

Depending on the phase, the patient may present with

Pathology

Autoimmune hepatitis may encompass multiple different conditions. 

Type 1: 

  • "classic" autoimmune hepatitis
  • ANA, IgG4, and anti-smooth muscle antibody may be elevated, with the last (1:320) a relatively more specific finding in type 1
  • ANCA is more often found in type 1 than in type 2

Type 2:

  • has antibodies to liver/kidney microsomes (ALKM-1) or to a liver cytosolic antigen (ALC-1)

Biopsy and histopathology is less specific for a diagnosis, compared to serum markers.

Radiographic features

The role of imaging in a patient with possible autoimmune hepatitis is threefold:

  • to exclude other possibilities of chronic hepatitis
  • to look for complications of cirrhosis (such as HCC)
  • to look for autoimmune involvement of other organs (such as the pancreas). 

CT

Non-specific, ranging from normal to hepatomegaly and cirrhosis.

MRI

Treatment and prognosis

Treatment is similar to other autoimmune conditions and often uses immunomodulating medications.

Differential diagnosis

  • -<p><strong>Autoimmune hepatitis</strong> (<strong>AIH</strong>) (plural: autoimmune hepatitides) is a rare type of <a href="/articles/chronic-hepatitis">chronic hepatitis</a>, currently classified as "type 1" or "type 2". It may eventually lead to <a href="/articles/cirrhosis">cirrhosis</a>. The role of imaging is primarily to exclude other diagnoses and evaluate for complications.</p><h4>Epidemiology</h4><p>It may occur in children or adults, but most patients are affected at 40-60 years old. The incidence in Western Europe is ~0.01-0.02% <sup>2</sup>.</p><p>There is no predilection for an ethnic group. There is a female predominance: 3.6: 1.</p><h4>Clinical presentation</h4><p>Autoimmune hepatitis has a variable clinical presentation. Patients may be asymptomatic or present in <a href="/articles/acute-liver-failure">acute liver failure</a> (rare), or anywhere between. It tends to have a relapsing-remitting course. There is an association with other autoimmune conditions.</p><p>Depending on the phase, the patient may present with</p><ul>
  • +<p><strong>Autoimmune hepatitis</strong> (<strong>AIH</strong>) (plural: autoimmune hepatitides) is a rare type of <a href="/articles/chronic-hepatitis">chronic hepatitis</a>, currently classified as "type 1" or "type 2". It may eventually lead to <a href="/articles/cirrhosis">cirrhosis</a>. The role of imaging is primarily to exclude other diagnoses and evaluate for complications.</p><p>Epidemiology</p><p>It may occur in children or adults, but most patients are affected at 40-60 years old. The incidence in Western Europe is ~0.01-0.02% <sup>2</sup>.</p><p>There is no predilection for an ethnic group. There is a female predominance: 3.6: 1.</p><p>Clinical presentation</p><p>Autoimmune hepatitis has a variable clinical presentation. Patients may be asymptomatic or present in <a href="/articles/acute-liver-failure">acute liver failure</a> (rare), or anywhere between. It tends to have a relapsing-remitting course. There is an association with other autoimmune conditions.</p><p>Depending on the phase, the patient may present with</p><ul>
  • -</ul><h4>Pathology</h4><p>Autoimmune hepatitis may encompass multiple different conditions. </p><p>Type 1: </p><ul>
  • +</ul><p>Pathology</p><p>Autoimmune hepatitis may encompass multiple different conditions. </p><p>Type 1: </p><ul>
  • -</ul><p>Type 2:</p><ul><li>has antibodies to liver/kidney microsomes (ALKM-1) or to a liver cytosolic antigen (ALC-1)</li></ul><p>Biopsy and histopathology is less specific for a diagnosis, compared to serum markers.</p><h4>Radiographic features</h4><p>The role of imaging in a patient with possible autoimmune hepatitis is threefold:</p><ul>
  • +</ul><p>Type 2:</p><ul><li>has antibodies to liver/kidney microsomes (ALKM-1) or to a liver cytosolic antigen (ALC-1)</li></ul><p>Biopsy and histopathology is less specific for a diagnosis, compared to serum markers.</p><p>Radiographic features</p><p>The role of imaging in a patient with possible autoimmune hepatitis is threefold:</p><ul>
  • -</ul><h5>CT</h5><p>Non-specific, ranging from normal to hepatomegaly and cirrhosis.</p><h5>MRI</h5><ul>
  • +</ul><p>CT</p><p>Non-specific, ranging from normal to hepatomegaly and cirrhosis.</p><p>MRI</p><ul>
  • -</ul><h4>Treatment and prognosis</h4><p>Treatment is similar to other autoimmune conditions and often uses immunomodulating medications.</p><h4>Differential diagnosis</h4><ul>
  • +</ul><p>Treatment and prognosis</p><p>Treatment is similar to other autoimmune conditions and often uses immunomodulating medications.</p><p>Differential diagnosis</p><ul>

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