Idiopathic pulmonary fibrosis

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]


quiz

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1 {Idiopathic pulmonary fibrosis (IPF) is characterized by _________.

alveolar epithelial cells apoptosis, fibroblast proliferation, and ECM protein deposition
swelling and scarring of the alveoli and interstitial tissues
presence of bacteria and other micro-organisms
viral-like illness

2 Which virus is sometimes associated with the onset of IPF?

Influenza virus
Epstein-Barr virus
Rhinovirus
Coronavirus

3 The 6A(4) allele of the SPA1 gene is associated with increased susceptibility to IPF in _________.

both smokers and nonsmokers
nonsmokers but not in smokers
only smokers
neither smokers nor nonsmokers


References

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  1. http://ajplung.physiology.org/content/295/3/L451.long
  2. http://www.lung.ca/diseases-maladies/a-z/pfibrosis-fibrosep/index_e.php
  3. http://ajplung.physiology.org/content/295/3/L451.long
  4. http://www.ncbi.nlm.nih.gov/omim/178500