Idiopathic pulmonary fibrosis

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Idiopathic pulmonary fibrosis (IPF) is a refractory and lethal interstitial lung disease characterized by alveolar epithelial cells apoptosis, fibroblast proliferation, and ECM protein deposition. [1] Pulmonary fibrosis is a chronic disease that causes swelling and scarring of the alveoli (air sacs) and interstitial tissues (tissue between cells) of the lungs. Bacteria and other micro-organisms are not routinely found in the lungs of patients with idiopathic pulmonary fibrosis. On the other hand, the condition does sometimes appear to follow a viral-like illness [2] particularly Epstein-Barr virus infection. [3] The 6A(4) allele of the SPA1 gene has been associated with an increased susceptibility to idiopathic pulmonary fibrosis in nonsmokers but not in smokers. [4]

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