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Hypersensitivity pneumonitis that has progressed to advanced fibrosis can be difficult to distinguish from idiopathic pulmonary fibrosis. Physiologic Consequences of Interstitial Lung Disease and Pulmonary Fibrosis The interstitial and intra-alveolar inflammation and scarring directly impairs the lungs ability to oxygenate the red blood cells. Oxygenation of the blood may remain normal at rest until the disease is far advanced. Stair and hill climbing, particularly while carrying heavy objects, are often the first noticeable symptoms of the disease. When pulmonary fibrosis progresses to an advanced stage, pulmonary hypertension may develop. This means the pressure in the system of blood vessels supplied by blood flow from the right-side of the heart to the vessels in the lungs may increase, particularly during exercise. If the average pulmonary arterial pressure exceeds 30 mm Hg (35 mm Hg with exercise), then pulmonary hypertension is said to be present. As pulmonary hypertension progresses, it can lead to right-sided heart enlargement and right heart failure. One of the major symptoms of right heart failure is swelling of the feet, ankles and legs. If the fibrosis progresses to a point where respiratory failure occurs, death is the usual outcome. Intravenous contrast does not aid in the diagnosis of interstitial lung disease, but can be very useful for diagnosis of pulmonary emboli. Certain diseases such as sarcoidosis and hypersensitivity pneumonitis tend to have the abnormalities distributed in a pattern that corresponds to bronchovascular bundles, the conglomeration of airways and attendant blood vessels and lymphatics. Hypersensitivity pneumonitis often is skewed toward a more upper-lobe distribution. In sharp contrast, idiopathic pulmonary fibrosis has a characteristic basilar (lower lung) distribution of disease. Ground glass opacities are characteristically seen in hypersensitivity pneumonitis, nonspecific interstitial pneumonia and during the acute exacerbation phase of idiopathic pulmonary fibrosis.

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