Anti-liver cytosolic antigen type 1 (LC1) antibodies in childhood autoimmune liver disease.
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 alpha 1-anti-trypsin deficiency [alpha 1-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.(ABSTRACT TRUNCATED AT 250 WORDS)
Han
S
S
Department of Immunology, King's College School of Medicine and Dentistry, London, England.
Tredger
M
M
Gregorio
G V
GV
Mieli-Vergani
G
G
Vergani
D
D
D000293:Adolescent; D000328:Adult; D000818:Animals; D001323:Autoantibodies / Q000032:analysis*; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D005455:Fluorescent Antibody Technique; D006801:Humans; D015151:Immunoblotting; D005779:Immunodiffusion; D007223:Infant; D008107:Liver Diseases / Q000276:immunology*; D008297:Male; D011817:Rabbits; D051381:Rats; D017208:Rats, Wistar; D013045:Species Specificity
D001323:Autoantibodies; D001324:Autoantigens; C077285:liver cytosol antigen type 1, human; C484108:liver cytosol antigen type 1, rat