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Idiopathic pulmonary fibrosis (IPF) synonymous with cryptogenic fibrosing alveolitis [5] is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue.
But pulmonary fibrosis can also appear without any known cause. In that case, it is termed "idiopathic". [7] Most idiopathic cases are diagnosed as idiopathic pulmonary fibrosis. This is a diagnosis of exclusion of a characteristic set of histologic/pathologic features known as usual interstitial pneumonia (UIP). In either case, a growing body ...
Since the medical term for conditions of unknown cause is "idiopathic", the clinical term for UIP of unknown cause is idiopathic pulmonary fibrosis (IPF). [2] Examples of known causes of UIP include connective tissue diseases (primarily rheumatoid arthritis ), drug toxicity, chronic hypersensitivity pneumonitis , asbestosis and Hermansky ...
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]
Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia [1] are a class of diffuse lung diseases. These diseases typically affect the pulmonary interstitium , although some also have a component affecting the airways (for instance, cryptogenic organizing pneumonitis ).
Pirfenidone, sold under the brand name Pirespa among others, is a medication used for the treatment of idiopathic pulmonary fibrosis.It works by reducing lung fibrosis through downregulation of the production of growth factors and procollagens I and II.
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