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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 ...
Idiopathic pulmonary fibrosis is interstitial lung disease for which no obvious cause can be identified (idiopathic) and is associated with typical findings both radiographic (basal and pleural-based fibrosis with honeycombing) and pathologic (temporally and spatially heterogeneous fibrosis, histopathologic honeycombing, and fibroblastic foci).
Pulmonary fibrosis is perpetuated by aberrant wound healing, rather than chronic inflammation. [15] It is the main cause of restrictive lung disease that is intrinsic to the lung parenchyma. In contrast, quadriplegia [ 16 ] and kyphosis [ 17 ] are examples of causes of restrictive lung disease that do not necessarily involve pulmonary fibrosis.
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).
Restrictive lung diseases are a category of extrapulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion, [2] resulting in a decreased lung volume, an increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary function test demonstrates a decrease in the forced vital capacity.
Bird fancier's lung (BFL), also known as bird breeder's lung, is a type of hypersensitivity pneumonitis.It can cause shortness of breath, fever, dry cough, chest pain, anorexia and weight loss, fatigue, and progressive pulmonary fibrosis (the most serious complication).
Occupational lung diseases comprise a broad group of diseases, including occupational asthma, industrial bronchitis, chronic obstructive pulmonary disease (COPD), bronchiolitis obliterans, inhalation injury, interstitial lung diseases (such as pneumoconiosis, hypersensitivity pneumonitis, lung fibrosis), infections, lung cancer and mesothelioma.
There is very limited data on treatment and follow-up of smoking-related interstitial fibrosis. One study reported that corticosteroids were not beneficial. Smoking cessation resulted in stable/non-progressive disease after several years of follow-up. Improvements in pulmonary function tests with smoking cessation have also been documented. [7]
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