Clinical and immunological heterogeneity of anti-liver-kidney microsome antibody-positive autoimmune hepatitis in children.
A group of children with autoimmune hepatitis is characterized by the presence in their sera of anti-liver-kidney microsome antibody (LKMA) as defined by immunofluorescence. Immunoblot analysis of the sera of 21 such children using rat-liver microsome total proteins as antigen allowed separation into three groups--group 1, whose sera recognized a 50 kDa protein; group 2, whose sera recognized a 66 kDa protein; and group 3, whose sera recognized both proteins. Patients with the anti-66-kDa reactivity more often displayed an acute onset of the disease, less signs of portal hypertension, better sensitivity to immunosuppressive therapy, and less tendency to relapse. They also displayed a lower titer of anti-rat-liver microsome antibody in enzyme-linked immunosorbent assay (ELISA) and a serum reactivity with a rat-liver cytosolic protein. These results (a) indicate that the LKMA-positive autoimmune hepatitis of children is heterogeneous from both clinical and immunological view points, (b) suggest that children with anti-66-kDa reactivity could have a less severe disease than children with the 50-kDa reactivity, and (c) indicate that immunoblot analysis should be added to ELISA and immunofluorescence studies to achieve better characterization of these patients.
Codoñer-Franch
P
P
Unité de Recherches (INSERM U 56), Hôpital de Bicêtre, France.
Bernard
O
O
Maggiore
G
G
Alagille
D
D
Alvarez
F
F
D000293:Adolescent; D001323:Autoantibodies / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005455:Fluorescent Antibody Technique; D006505:Hepatitis / Q000276:immunology*; D006801:Humans; D007223:Infant; D007668:Kidney / Q000276:immunology*; D008297:Male; D008862:Microsomes, Liver / Q000276:immunology*; D012189:Retrospective Studies
D001323:Autoantibodies