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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 ] Complications may include pulmonary hypertension , respiratory failure , pneumothorax , and lung cancer .
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.
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.
Fibrosis can occur in many tissues within the body, typically as a result of inflammation or damage. Common sites of fibrosis include the lungs, liver, kidneys, brain, and heart: Micrograph showing cirrhosis of the liver. The tissue in this example is stained with a trichrome stain, in which fibrosis is colored blue.
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]
Generally, intrinsic causes are from lung parenchyma diseases that cause inflammation or scarring of the lung tissue, such as interstitial lung disease or pulmonary fibrosis, or from having the alveoli air spaces filled with external material such as debris or exudate in pneumonitis. [3]
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