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Liver infiltrating mononuclear cells in children with type 1 autoimmune hepatitis

¹ División inmunogenética, Hospital de Clínicas "José de San Martín", Universidad de Buenos Aires, Buenos Aires, Argentina
² Anatomía patológica, Hospital de Clínicas "José de San Martín", Universidad de Buenos Aires
³ Sección Anatomía patológica, Hospital Nacional de Pediatría "J P Garrahan", Buenos Aires
⁴ Hepatología, Hospital Nacional de Pediatría "J P Garrahan", Buenos Aires
⁵ Transplante hepático, Hospital Nacional de Pediatría "J P Garrahan", Buenos Aires
⁶ Unidad de Anatomía patológica, Hospital Nacional de Pediatría "J P Garrahan", Buenos Aires
⁷ Hepatología, Hospital Nacional de Pediatría "J P Garrahan", Buenos Aires
⁸ Unidad de Hepatología, Hospital municipal de niños de San Justo, Buenos Aires, Argentina
⁹ Unidad de Hepatología, Hospital general de infecciosas "F J Muñiz", Buenos Aires
¹⁰ Unidad de Hepatología, Hospital de Gastroenterología "Dr C Bonorino Udaondo", Buenos Aires

Correspondence to:
Dr A C Cherñavsky
División Inmunogenética, Hospital de Clínicas "José de San Martín", Universidad de Buenos Aires, Av Córdoba 2351, (1120) Buenos Aires, Argentina; accher{at}fibertel.com.ar 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.

Abbreviations: 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

Keywords: type 1 autoimmune hepatitis; immunohistochemistry; mononuclear cell infiltrate; children