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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]
12 per 100,000 people per year [4] 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.
Nintedanib is used for the treatment of idiopathic pulmonary fibrosis. [9] It has been shown to slow down decrease in forced vital capacity, [10][11] and it also improves people's quality of life. [12] Nintedanib does not improve survival in people with IPF. [13]
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.
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. [7] ILD is associated with a 3-fold increased risk of lung cancer. [7]
Prognosis is related to the underlying disorder and the type and severity of lung disease. In severe cases, lung transplantation can be considered. This is more common in cases of bronchiolitis obliterans, pulmonary fibrosis, or pulmonary hypertension. Most complications are not fatal, but does reduce life expectancy to an estimated 5 to 10 ...
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