Objective: To investigate infiltrating cells in the liver of children with type 1 autoimmune hepatitis (AH-1).
Methods: liver biopsies from 24 untreated AH-1 patients (14 children, 10 adults), five patients with hepatitis C virus related chronic hepatitis (HCV), and 10 control liver specimens (CL) were processed for immunohistochemical cell characterisation.
Results: Two different cell distribution patterns were detected in the liver of patients with AH-1: (1) CD4+ and CD20+ cells were found in the central areas of the portal tracts (portal distribution); (2) CD8+ cells were observed at the periphery of the portal space (periportal distribution). Some cell subsets, like CD56, CD57, Fas-L, and Bak, showed a non-defined distribution pattern. The presence of two well defined patterns of cell distribution was not observed in HCV and CL (CD4+, CD20+, and CD8+ cells were uniformly distributed in the portal space). In AH-1 and CL, the NK markers CD56 and CD57 were found scattered throughout the liver parenchyma. However, in HCV biopsies, CD56+ cells were also clearly increased in both the portal and the periportal areas. Biopsies of AH-1 and HCV patients showed a uniform distribution of Fas-L and Bak in the portal and periportal areas, with Bak staining also detected in the hepatic parenchyma.
Conclusions: Despite clinical and genetic differences, there was a similar distribution of liver infiltrating mononuclear cells in children and adults with AH-1. These results raise the possibility of reclassifying cryptogenic chronic hepatitis by immunohistochemical analysis of infiltrating liver cells.
- AAH, adult autoimmune hepatitis type 1
- AH-1, type 1 autoimmune hepatitis
- ANA, antinuclear antibodies
- CL, control liver specimen
- HCV, hepatitis C virus related chronic hepatitis
- PAH, paediatric autoimmune hepatitis type 1
- SMA, smooth muscle antibodies
- type 1 autoimmune hepatitis
- mononuclear cell infiltrate
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