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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
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 ...
Well-demarcated, nodular lesions ranging 2–5 mm in pulmonary parenchyma. Type II pneumocytes without nuclear atypia lined thickened alveolar septa and proliferated papillary structures. Enlarged cuboidal cells lining mildly thickened alveolar septa. [11] Enlarged cuboidal cells have abundant, eosinophilic cytoplasm and large, round nuclei. [12]
Pulmonary capillary hemangiomatosis (PCH) is a disease affecting the blood vessels of the lungs, where abnormal capillary proliferation and venous fibrous intimal thickening result in progressive increase in vascular resistance. [1] It is a rare cause of pulmonary hypertension, and occurs predominantly in young adults.
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.
Overall, about 50% develop permanent pulmonary abnormalities, and 5 to 15% have progressive fibrosis of the lung parenchyma. Sarcoidosis of the lung is primarily an interstitial lung disease in which the inflammatory process involves the alveoli, small bronchi, and small blood vessels. [29]
The onset of pulmonary hemorrhage is characterized by a cough productive of blood and worsening of oxygenation leading to cyanosis. [1] Treatment should be immediate and should include tracheal suction, oxygen, positive pressure ventilation , and correction of underlying abnormalities such as disorders of coagulation .
Preservation of lung volumes in the presence of increased interstitial markings is a radiographic hallmark of LAM that helps distinguish it from most other interstitial lung diseases, in which alveolar septal and interstitial expansion tend to increase the lung's elastic recoil properties and decreased lung volumes.
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