The three main novel observations of this study are that: (1) abnormalities in both the innate and adaptive immune response can be detected in the circulating blood of patients with IPF at diagnosis; (2) some of these abnormalities relate to the severity of lung function at diagnosis; and, (3) a specific and longitudinally stable immune phenotype characterized by increased NKT-like cells, CD8+ T cells with an exhausted phenotype, and less naïve T cells, with an impaired CD4/CD8 ratio, is associated with IPF progression over time (AUC 0.94), despite the use of antifibrotic treatment. Here, CD8A is linked to idiopathic pulmonary fibrosis.