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Idiopathic pulmonary fibrosis (IPF) synonymous with cryptogenic fibrosing alveolitis[5] is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue.
Pulmonary fibrosis. Pulmonary fibrosis is a condition in which the lungs become scarred over time. [1] Symptoms include shortness of breath, a dry cough, feeling tired, weight loss, and nail clubbing. [1] Complications may include pulmonary hypertension, respiratory failure, pneumothorax, and lung cancer. [2]
Progressive massive fibrosis (PMF), characterized by the development of large conglomerate masses of dense fibrosis (usually in the upper lung zones), can complicate silicosis [1] and coal worker's pneumoconiosis. [2] Conglomerate masses may also occur in other pneumoconioses, such as talcosis, [3] berylliosis (CBD), [3] kaolin pneumoconiosis ...
Specialty. Respirology. Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. [1] The scarring (pulmonary fibrosis) involves the pulmonary interstitium (the supporting framework of the lung). UIP is thus classified as a form of interstitial lung disease.
Interstitial lung disease. Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), [3] is a group of respiratory diseases affecting the interstitium (the tissue) and space around the alveoli (air sacs) of the lungs. [4] It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and ...
It received U.S. Food and Drug Administration (FDA) approval for use for idiopathic pulmonary fibrosis in 2014 – one of only two drugs available for treating IPF – and numerous studies since have demonstrated its effectiveness in slowing the progressive, terminal lung disease. [8]
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