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Such scar tissue causes an irreversible decrease in oxygen diffusion capacity, and the resulting stiffness or decreased compliance makes pulmonary fibrosis a restrictive lung disease. [14] Pulmonary fibrosis is perpetuated by aberrant wound healing, rather than chronic inflammation. [15] It is the main cause of restrictive lung disease that is ...
It is a type of interstitial lung disease. [3] People often benefit from pulmonary rehabilitation and supplemental oxygen. [1] Certain medications like pirfenidone or nintedanib may slow the progression of the disease. [2] Lung transplantation may also be an option. [1] About 5 million people are affected globally. [8]
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. It ...
The differential diagnosis includes other types of lung disease that cause similar symptoms and show similar abnormalities on chest radiographs. Some of these diseases cause fibrosis, scarring or honeycomb change. The most common considerations include: chronic hypersensitivity pneumonitis; non-specific interstitial pneumonia; sarcoidosis
These patients were all heavy smokers and considered to have a form of interstitial lung disease. Most were in their 40s and had abnormalities in pulmonary function tests, most commonly reduced diffusion capacity for carbon monoxide (DLCO). Their symptoms generally remained stable (did not worsen) over up to 10 years of follow-up. [7]
While patchy bilateral disease is typical, there are unusual variants of organizing pneumonia where it may appear as multiple nodules or masses. One rare presentation, focal organizing pneumonia, may be indistinguishable from lung cancer based on imaging alone, requiring biopsy or surgical resection to make the diagnosis. [20]
This includes fungal infections, mycobacterial infections such as tuberculosis or mycobacterium avium intracellulare, bronchopneumonia, chronic aspiration pneumonia, cystic fibrosis or cellular impaction from bronchovascular spread of malignancy, as can occur with breast cancer, leukemia or lymphoma. [2]
The term fibrothorax implies severe fibrosis affecting both the visceral and the outer (parietal) pleura, fusing the lung to the chest wall. [2] The condition starts as an undrained pleural effusion. Over time, the undrained pleural effusion causes sustained inflammation of the pleura, which can then lead to deposition of fibrin in the pleura ...