Altogether, our integrative analyses of circulating self-antigen-specific and dominant intrahepatic CD4 T cell clones in AILD patients converge to establish that some autoreactive CD4 T cell clones can be found both in the liver and in the blood, where they are defined as PD-1+CXCR5−HLA-DR+ mCD4 T cells, with a specific autoimmune and immuno-exhausted transcriptional profile related to an active immune response in the tissue. Here, PIGN is linked to angioimmunoblastic T-cell lymphoma.