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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]
Fibrosis can affect one or both of the two layers of tissue forming the pleura—the visceral pleura adjacent to the lung and the parietal pleura adjacent to the ribcage. The term fibrothorax implies severe fibrosis affecting both the visceral and the outer (parietal) pleura, fusing the lung to the chest wall. [2]
Idiopathic pulmonary fibrosis; Other names: Fibrosing alveolitis, cryptogenic fibrosing alveolitis, diffuse fibrosing alveolitis, usual interstitial pneumonitis, diffuse interstitial pneumonitis: Figure A shows the location of the lungs and airways in the body. The inset image shows a detailed view of the lung's airways and air sacs in cross ...
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]
Pulmonary rehabilitation is generally specific to the individual patient, with the objective of meeting the needs of the patient. It is a broad program and may benefit patients with lung diseases such as chronic obstructive pulmonary disease (COPD), sarcoidosis, idiopathic pulmonary fibrosis (IPF) and cystic fibrosis, among others.
The median survival in idiopathic pulmonary fibrosis is 3-3.5 years. In those who receive a lung transplant, the medial survival in idiopathic pulmonary fibrosis is 5.2 years, as compared to 6.7 years in those with other types of ILD. [8] ILD is associated with a 3-fold increased risk of lung cancer. [8]
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