Diagnosis, treatment, and follow-up of autoimmune hepatitis

Autoimmune Hepatitis Clinical Practice Guidelines (2019)

The guidelines on the diagnosis, treatment, and follow-up of autoimmune hepatitis were released on January 1, 2019, by the Hellenic Association for the Study of the Liver.

Clinical Characteristics

Autoimmune hepatitis (AIH) should be considered a possibility in any individual with acute or chronic hepatitis, particularly in those with high immunoglobulin G (IgG) levels.

Untreated AIH carries high morbidity and mortality rates, so early and accurate diagnosis is mandatory.

Screening for concomitant autoimmune diseases can be considered in patients with AIH (particularly autoimmune thyroiditis), as AIH is associated with various other autoimmune-mediated conditions.

Cirrhosis should be suspected upon AIH diagnosis, since nearly one-third of adults and half of children with AIH are at the stage of cirrhosis at AIH diagnosis.

The presentation of acute AIH can take one of two clinical forms, as follows:

• Acute worsening of previously undiagnosed or misdiagnosed AIH
• Original acute-onset AIH without chronic lesions on liver histology

AIH can be classified as either (1) AIH-1, antinuclear (ANA), smooth muscle (SMA), and/or soluble liver antigens/liver pancreas antibody (anti-SLA/LP)–positive or (2) AIH-2, anti-liver/kidney microsomal antibody type-1 (anti-LKM1), anti-liver/kidney microsomal antibody type-3 (anti-LKM3), and/or liver cytosol type-1 antigen (anti-LC1)–positive.

Complications

Patients with AIH-related cirrhosis should undergo ultrasonography every 6 months to monitor for hepatocellular carcinoma (HCC).

Workup

All children with AIH should undergo magnetic resonance cholangiopancreatography (MRCP) at minimum to rule out autoimmune sclerosing cholangitis.

Patients who have AIH with cholestatic features should undergo testing for primary biliary cholangitis (PBC).

Interface hepatitis, hepatocyte rosetting, and emperipolesis strongly support but do not confirm AIH.

In daily clinical practice, the 2008 simplified score should be used for AIH diagnosis.

The 1999 revised score can help diagnose difficult AIH cases, since it incorporates treatment response as an important parameter.

Diagnosis of AIH-PBC and AIH–primary sclerosing cholangitis (PSC) variants should not be based on diagnostic scores, but they may be used cautiously in the diagnosis of childhood AIH and acute or fulminant disease.

Treatment

The goal of AIH therapy is to achieve complete biochemical and histological remission to prevent disease progression.

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