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Hypoxia caused by pulmonary fibrosis can lead to pulmonary hypertension, which in turn can lead to heart failure of the right ventricle. Hypoxia can be prevented by oxygen supplementation. [3] Pulmonary fibrosis may also result in an increased risk of pulmonary emboli, which can be prevented by anticoagulants. [3]
Usually, the underlying condition has to cause intense inflammation of the pleura, though it is unclear exactly how this results in fibrosis. The precise mechanisms producing the fibrosis are not entirely clear. However, research indicates a protein called Transforming Growth Factor beta plays a central role in producing fibrothorax. [3]
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 ...
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.
Pamrevlumab (INN; [1] development code FG-3019) is a humanized monoclonal antibody designed for the treatment of idiopathic pulmonary fibrosis and pancreatic cancer. It binds to the connective tissue growth factor (CTGF) protein. [2] This drug was developed by FibroGen, Inc. [3]
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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