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Pulmonary fibrosis is associated with the increased formation of connective tissue in the lungs, resulting in scarring fibrosis of functional lung tissue. This leads to a decrease in the inner surface of the extremely fine alveoli and the extensibility of the lungs, which, in turn, impedes the intake of oxygen and the release of carbon dioxide. The result is impaired lung function. IPF is a particularly aggressive form of the disease that cannot be attributed to a specific cause. The symptoms worsen rapidly.

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