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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 .
It is a type of chronic pulmonary fibrosis characterized by a progressive and irreversible decline in lung function. [6] [3] [4] The tissue in the lungs becomes thick and stiff, which affects the tissue that surrounds the air sacs in the lungs. [7] Symptoms typically include gradual onset of shortness of breath and a dry cough. [1]
Fibrosis can affect one or both of the two layers of tissue forming the pleura—the visceral pleura adjacent to the lung and the parietal pleura adjacent to the ribcage. The term fibrothorax implies severe fibrosis affecting both the visceral and the outer (parietal) pleura, fusing the lung to the chest wall. [2]
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]
Infant respiratory distress syndrome due to a deficiency of surfactant in the lungs of a baby born prematurely. Tuberculosis [4] Many cases of restrictive lung disease are idiopathic (have no known cause). Still, there is generally pulmonary fibrosis. [5] Examples are: Idiopathic pulmonary fibrosis
Prognosis is related to the underlying disorder and the type and severity of lung disease. In severe cases, lung transplantation can be considered. This is more common in cases of bronchiolitis obliterans, pulmonary fibrosis, or pulmonary hypertension. Most complications are not fatal, but does reduce life expectancy to an estimated 5 to 10 ...
Pulmonary function: increased residual volume, increased total lung capacity, fixed obstruction, low diffusing capacity of the lung for carbon monoxide that corrects with alveolar volume; High-resolution CT scan: diffuse pulmonary nodules 4–10 mm, greater than 20 nodules, mosaic attenuation or air trapping in greater than 50% of the lung
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
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