Rare autoimmune diseases of the liver

scheme for serological differentiation of type 1 AIH and type 2 autoimmune hepatitisThe reason for my renewed interest is that we brought four more ELISA tests for liver diagnostics to the market two weeks ago. They are the Anti-LKM-1, Anti-SLA, Anti-gp210, and Anti-Sp100 tests, all designed for fully automated autoimmune diagnosis with our Alegria system. All four test systems assist the formulation of a diagnosis when autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) are suspected, or for differential diagnosis when another disorder of the liver is assumed.

Autoimmune diseases of the liver

The requirements for successful treatment of liver disease naturally include a precise diagnosis. As a result, unexplained elevated liver enzymes generally lead the treating physician on a complicated search for the source of the abnormally elevated liver values. In addition to anamnesis and clinical findings, laboratory tests and the correct interpretation of the laboratory results play a critical role in this process.

If the doctor is able to rule out alcohol abuse, drug-induced hepatitis, and viral hepatitis (for example hepatitis B, C, or D), and if nothing points to hemochromatosis, alpha-1-antitrypsin deficiency, or Wilson’s disease (also called: hepatolenticular degeneration), the most likely suspects remaining are autoimmune liver diseases.

This scheme shows relevant autoantibodies for serodiagnosing autoimmune liver diseases.In cases of autoimmune liver disease, the body loses its ability to tolerate its own hepatocellular or cholangiocellular tissues in the liver. The causes of these diseases remain a mystery.

When it comes to autoimmune diseases of the liver, there are essentially three conditions to differentiate:

  1. autoimmune hepatitis (AIH), of which there are two variants, type 1 AIH and type 2 AIH.
  2. primary biliary cirrhosis (PBC)
  3. primary sclerosing cholangitis (PSC)

The individual clinical presentations of these diseases may overlap, which is what makes the appraisal and interpretation of laboratory values, as well as the formation of a diagnosis, so difficult for these autoimmune diseases. Overlap syndromes are particularly common between autoimmune hepatitis and PBC; overlap between the autoimmune liver diseases and viral hepatitis C is also not uncommon. (Here you will find a .)

Autoimmune hepatitis (AIH)

Type 1 autoimmune hepatitis (type 1 AIH) is considered “classic” autoimmune hepatitis. This disorder mostly occurs in young women and is typically characterized by the presence of antinuclear antibodies (ANA) and/or anti-smooth muscle antibodies (ASMA, or: SMA), with F-actin generally acting as the target antigen. Autoantibodies against the soluble liver antigen/liver pancreas antigen (anti-SLA/LP antibodies) are often also detected. Depending on the study, this is the case in up to 25% of affected patients.

In the past, this form of “SLA-positive AIH” was considered to be its own variant of autoimmune hepatitis and was formerly known as “type 3 autoimmune hepatitis” (“type 3 AIH”). Because this anti-SLA positive form of the disease is clinically and therapeutically no different from type 1 autoimmune hepatitis, the differentiation between type 1 and type 3 AIH was abandoned. Clearly different from type 1 AIH, type 2 AIH is primarily characterized by the presence of anti-LKM-1 and anti-LC-1 antibodies.

The autoantibodies typically detected in cases of autoimmune hepatitis are: antinuclear antibodies (ANA), anti-smooth muscle antibodies (ASMA), LKM-1 antibodies, LC-1 antibodies, and anti-SLA/LP antibodies.

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