Progressive pulmonary fibrosis

Progressive pulmonary fibrosis is a clinical phenotype characterized by the relentless worsening of fibrotic lung disease in patients with an interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis It affects the respiratory system across diverse populations, occurring as a complication of varied conditions including autoimmune or connective tissue diseases, fibrotic hypersensitivity pneumonitis, idiopathic nonspecific interstitial pneumonia, and unclassifiable ILDs

Progressive pulmonary fibrosis is a clinical phenotype characterized by the relentless worsening of fibrotic lung disease in patients with an interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis It affects the respiratory system across diverse populations, occurring as a complication of varied conditions including autoimmune or connective tissue diseases, fibrotic hypersensitivity pneumonitis, idiopathic nonspecific interstitial pneumonia, and unclassifiable ILDs Pathophysiol...

Pathophysiologically, it features self-sustaining alveolar architecture destruction, fibroproliferation, and excessive extracellular matrix deposition

Clinically, it is defined by the occurrence of at least two of three criteria within the preceding year without an alternative explanation: worsening respiratory symptoms, physiological disease progression (such as absolute decline in forced vital capacity or gas transfer), and radiologic evidence of disease progression on high-resolution computed tomography

Clinically, it is defined by the occurrence of at least two of three criteria within the preceding year without an alternative explanation: worsening respiratory symptoms, physiological disease progression (such as absolute decline in forced vital capacity or gas transfer), and radiologic evidence of disease progression on high-resolution computed tomography