Chronic respiratory failure

Abstract

Idiopathic pulmonary fibrosis (IPF) is a form of interstitial lung disease that may be worsened by chronic respiratory failure (CRF) as the disease progresses into advanced stages. Many pathophysiological mechanisms underlying CRF occur with IPF over time, including reduced lung compliance and volume from airway obstruction and capillary surface destruction, resulting in impaired diffusion capacity and ventilation-perfusion mismatch. These complex physiological changes affect all lung functions and compartments and worsen the underlying disease. Consequently, in the majority of these cases, CRF develops as a secondary cause of IPF progression.