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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 ...
Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), [3] is a group of respiratory diseases affecting the interstitium (the tissue) and space around the alveoli (air sacs) of the lungs. [4] It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues.
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]
Patients with idiopathic pulmonary fibrosis were randomly assigned to treatment with oral pirfenidone or placebo for a minimum of 72 weeks. [47] In study 004, pirfenidone reduced decline in forced vital capacity. Mean change in FVC at week 72 was –8.0% in the pirfenidone 2403 mg/day group and –12.4% in the placebo group, a difference of 4.4%.
Pulmonary rehabilitation. Pulmonary rehabilitation, also known as respiratory rehabilitation, is an important part of the management and health maintenance of people with chronic respiratory disease who remain symptomatic or continue to have decreased function despite standard medical treatment. It is a broad therapeutic concept.
The list price of a year's treatment in the US is US$322,000 [44] (formerly US$311,000). [45] However, a 2020 report by Institute for Clinical and Economic Review found that the price has made the treatment not cost effective and that "an appropriate health-benefit price would range from $67,900–$85,500 per year". [46] [47]
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