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Anti‐liver cytosolic antigen type 1 (LC1) antibodies in childhood autoimmune liver disease
Author(s) -
Han Shuhua,
Tredger Micheal,
Gregorio Germana V.,
MieliVergani Giorgina,
Vergani Diego
Publication year - 1995
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.1840210111
Subject(s) - autoimmune hepatitis , ouchterlony double immunodiffusion , autoantibody , antibody , liver disease , immunodiffusion , medicine , immunology , pathology , antigen , hepatitis , biology , antiserum
Antibodies to liver cytosol antigen type 1 (anti‐LC1), which recognize a 60‐kd peptide contained in the liver cytosolic fraction, have been reported to define a subset of autoimmune hepatitis (AIH) either negative for other autoantibodies or positive for anti‐liver kidney microsomal antibody type 1 (LKM‐1) and to be best detected in immunodiffusion. To analyze the prevalence of anti‐LC1 in childhood liver disease, we have tested the sera of 95 patients using immunoblot, indirect immunofluorescence, and immunodiffusion. Fifteen children had smooth muscle antibody (SMA) and/or anti‐nuclear antibody (ANA)‐positive AIH, 13 had anti‐LKM‐1‐positive AIH, 14 had autoimmune sclerosing cholangitis (ASC) (all SMA and/or ANA positive), and 53 had non‐autoimmune liver disease (10 had α1‐anti‐trypsin deficiency [α1‐ATD], 11 had Wilson's disease [WD], 14 had Alagille's syndrome, and 18 had chronic hepatitis B virus [HBV] infection). Twenty healthy children were studied as controls. Anti‐LC1 positivity in immunodiffusion and strong reactivity in immunoblot were found in 4 LKM‐1‐ and 2 SMA/ANA‐positive patients with AIH and in 1 patient with ASC, but in none of the patients with other liver diseases nor in controls. A weak 60‐kd band was detected by immunoblot in 6 more patients with AIH (2 were LKM‐1‐ and 4 were SMA/ANA‐positive) and 6 patients with ASC, all anti‐LC1‐negative by immunofluorescence and immunodiffusion. No distinct clinical features characterized the anti‐LC1‐positive patients. Our data show that, in pediatric age, LC1 reactivity, although associated with autoimmune liver disease, does not identify a distinct disease, subset and that immunoblot is the most sensitive technique to detect anti‐LC1. (Hepatology 1995;21:58–62).