ABSTRACT
Autoimmune liver diseases (ALD) are characterized by immune-mediated injury of bile
ducts or hepatocytes, thus including cholangiopathies such as primary biliary cirrhosis
(PBC), primary sclerosing cholangitis, and autoimmune hepatitis. Further, ALD variants
manifesting with both hepatocellular and cholangiocellular damage are becoming more
common. Serum autoantibodies, together with imaging and histology, are critical to
the diagnostic process when ALD is suspected. Because an early diagnosis can influence
prognosis, the development of sensitive and specific tests for serum autoantibodies
should be a priority for researchers to ensure a more efficient noninvasive workup.
Little prognostic value has been observed for any of the ALD serum hallmarks, and
a vigorous effort to investigate new and old markers should therefore be undertaken
in longitudinal studies as in the recent paradigm of PBC-specific antinuclear antibodies.
We review herein the numerous ALD screening tests available in routine and specialized
laboratories and comment on their significance in clinical practice.
KEYWORDS
Autoimmune hepatitis - primary biliary cirrhosis - primary sclerosing cholangitis
- overlap syndromes - serum autoantibodies
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Pietro InvernizziM.D. Ph.D.
Division of Internal Medicine and Liver Unit, San Paolo Hospital School of Medicine,
University of Milan
Via di Rudinì 8, 20142 Milano, Italy