Peripheral blood T-cell responses to pyruvate dehydrogenase complex in primary biliary cirrhosis: role of antigen-presenting dendritic cells

Eur J Clin Invest. 2001 Jul;31(7):639-46. doi: 10.1046/j.1365-2362.2001.00847.x.


Patients with primary biliary cirrhosis (PBC) are usually characterized by the presence of antibody to pyruvate dehydrogenase complex (PDC) in the sera and PDC-specific T cells in the liver. However, most of the patients with PBC do not show peripheral blood T cells response to PDC. In this study, we re-evaluated the peripheral blood T cell responses to PDC in PBC using antigen-presenting dendritic cells (DCs). Twenty-four patients with PBC (AMA-positive: 16; AMA-negative: 8) and 13 normal controls were enrolled in the study. Peripheral blood mononuclear cells (PBMC) and highly enriched populations of T cells were stimulated with either only PDC or DCs plus PDC or PDC-pulsed DC plus PDC. Antibodies to different components of PDC were estimated by an immunoblotting technique. PBMC from only one out of ten AMA-positive PBC patients proliferated when cultured with only PDC. However, peripheral blood T cells from ten out of ten AMA-positive PBC patients and three out of ten AMA-negative PBC patients, but none of the five normal controls showed PDC-specific proliferation when cultured with PDC-pulsed DCs. Two of these three AMA-negative PBC patients, although negative for AMA, were positive for antibodies to other components of PDC. PDC-specific T cells are present in the peripheral blood from most of the patients with PBC. This is the first report on the effectiveness of antigen-pulsed DCs for the elucidation of autoantigen-specific immune response in human autoimmune diseases.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antigen Presentation
  • Autoantibodies / blood
  • Autoimmune Diseases / immunology*
  • Dendritic Cells / immunology*
  • Female
  • Humans
  • Liver Cirrhosis, Biliary / immunology*
  • Lymphocyte Activation
  • Male
  • Middle Aged
  • Mitochondria / immunology
  • Pyruvate Dehydrogenase Complex / immunology*
  • T-Lymphocyte Subsets / immunology*


  • Autoantibodies
  • Pyruvate Dehydrogenase Complex