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Idiopathic pulmonary fibrosis is a serious disease where the tiny air sacs of the lungs (the alveoli) and the lung tissue next to the alveoli become damaged and scarred. The main symptom is shortness of breath that gradually gets worse. The exact cause is not known. Idiopathic pulmonary fibrosis used to be called cryptogenic fibrosing alveolitis. The exact cause is not known (hence the term idiopathic). It was thought that inflammation within the alveoli played a big role in the development of IPF and that this inflammation led to scarring (fibrosis). This led to the name cryptogenic fibrosing alveolitis. However, treatments that help to reduce inflammation are not always effective. Therefore, the role of inflammation has recently been brought into question.
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