Decreased lung compliance + Lungs are stiffer and more resistant to expansion (Figure 9-8) + Lung compliance curve is shifted downward and to the right (Figure 9-20) + Static recoil pressure is increased at total lung capacity (TLC) (owing to increased elastic forces) = Work of breathing is increased Decreased lung volumes as a consequence of decreased lung compliance + TLC, vital capacity (VC), functional residual capacity (FRO), and residual volume (RV) are proportionally reduced + Tidal volumes are reduced (Figure 9-10) + Alveolar ventilation is maintained by increased respiratory rate (Figure 9-10) Disturbances in gas exchange + Patchy nature of fibrosis leads to severe inhomogeneity in ventilation + Regional inhomogeneity causes mismatching of ventilation and perfusion with shift toward low V/Q regions, including areas of absent ventilation (shunt) + Increased A-a APO, secondary to low regions, with hypoxemia in severe cases + Decreased pulmonary diffusing capacity (DLCO) owing to thickened alveolar walls and loss of pulmonary capillaries, leading toa reduction in pulmonary capillary surface area + Hypoxemia typically exacerbated by exercise (Fioure 9-24) + PaCO, typically normal or low owing to increased minute ventilation; hypercarbia in pure idiopathic pulmonary fibrosis (IPF) is indicative of very advanced disease Pulmonary artery hypertension + Decreased pulmonary capillary surface area + Increased pulmonary vascular resistance from reduced FRC + Inhomogeneity of ventilation causes regional alveolar hypoxia with consequent hypoxic pulmonary vasoconstriction + Marked exacerbation with exercise owing to inability to recruit new vessels