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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 ...
Treatment options for idiopathic pulmonary fibrosis are very limited, since no current treatment has stopped the progression of the disease. Because of this, there is no evidence that any medication can significantly help this condition, despite ongoing research trials. Lung transplantation is the only therapeutic option available in severe cases.
Pirfenidone, sold under the brand name Pirespa among others, is a medication used for the treatment of idiopathic pulmonary fibrosis.It works by reducing lung fibrosis through downregulation of the production of growth factors and procollagens I and II.
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]
Since the medical term for conditions of unknown cause is "idiopathic", the clinical term for UIP of unknown cause is idiopathic pulmonary fibrosis (IPF). [2] Examples of known causes of UIP include connective tissue diseases (primarily rheumatoid arthritis ), drug toxicity, chronic hypersensitivity pneumonitis , asbestosis and Hermansky ...
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]
Idiopathic pulmonary fibrosis causes the lungs to become scarred which results in a difficulty in breathing. The causes are of this disease is still not fully known, however, prevention methods include self-care such as stopping smoking and exercising.
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.
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