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2017 ; 6
(5
): e139
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English Wikipedia
Diagnostic autoantibodies for autoimmune liver diseases
#MMPMID28690845
Toh BH
Clin Transl Immunology
2017[May]; 6
(5
): e139
PMID28690845
show ga
Autoimmune liver diseases are conditions of low prevalence that comprise the
triad of autoimmune hepatitis, primary biliary cholangitis (cirrhosis) and
primary sclerosing cholangitis and their poorly characterised overlapping
syndromes. Diagnostic autoantibodies are associated with autoimmune hepatitis and
primary biliary cholangitis but not with primary sclerosing cholangitis.
Autoantibodies are useful disease markers that facilitate early diagnosis of
autoimmune hepatitis and primary biliary cholangitis and allow for therapeutic
intervention to prevent progression to liver cirrhosis and associated
complications. Adult onset type 1 autoimmune hepatitis is associated with F-actin
reactive smooth muscle autoantibody, antinuclear autoantibody in 60% of patients,
and autoantibody to SLA/LP in 15-20%. Juvenile onset type 2 autoimmune hepatitis
is associated with LKM-1 and LC-1 autoantibodies. Primary biliary cholangitis is
associated with a mitochondria-associated autoantibody designated M2 in >90% of
patients and with disease-specific antinuclear autoantibodies in 50% that bind to
antigens in the nuclear core complex and in multiple nuclear dots. Autoantibodies
to the nuclear core complex target gp210, nucleoporin p62 and nuclear lamin B
receptor. Autoantibodies to multiple nuclear dots target Sp100 and PML antigens.
Liver autoantibodies in asymptomatic patients with normal liver function may
precede the subsequent development of overt autoimmune liver disease. For routine
diagnostic immunology laboratories, initial screening for liver autoantibodies by
immunofluorescence remains the method of choice with confirmation for reactivity
with their target antigen by enzyme-linked immunosorbent assay (ELISA) or line
blot when required.