We have identified two distinct populations of CXCR4+ cell, one of epithelial origin (CXCR4+/e-cadherin+) and one of myeloid origin (CXCR4+/CD45+), that were increased in lung tissue of patients with IPF compared to non-diseased control (NDC) donors. Here, CXCR4 is linked to idiopathic pulmonary fibrosis.