A decreased frequency of the KIR-ligand combination KIR3DL1/HLA-Bw4 has also been found in patients with PSC [56] which furthermore confirms that KIRs and their HLA ligands are likely to have a contributory role in the immunological pathways underlying the development and progression of autoimmune liver disease. Here, KIR3DL1 is linked to autoimmune hepatitis.