Etiology
Despite extensive investigation, the cause of IPF remains unknown. The condition involves abnormal and excessive deposition of collagen (fibrosis) in the pulmonary interstitium (mainly the walls of the alveoli) with minimal associated inflammation. The fibrosis in IPF has been linked to cigarette smoking, gastroesophageal reflux disease and autoimmune disorders, but none of these are present in all patients with IPF, and therefore do not provide a completely satisfactory explanation for the disease.
Genetic associations include SFTPA1, SFTPA2, TERT, and TERC.
Read more about this topic: Idiopathic Pulmonary Fibrosis