Idiopathic pulmonary fibrosis risk factors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]


Common risk factors of pulmonary fibrosis include cigarette smoking and genetic mutations which include hTERT, MUC5B, TERT, and RTEL1. Less common risk factors include gastroesophageal reflux disease and wood dust.

Risk Factors

Common risk factors

  • Common risk factors of idiopathic pulmonary fibrosis include the following:
    • Cigarette smoking:[1]
      • Is the most important risk factor for idiopathic pulmonary fibrosis even years after cessation
    • Gene mutations:
      • Genetic mutations showed increase risk in development of pulmonary fibrosis. Mutations include the following genes:[2][3][4]
        • hTERT and hTR
        • MUC5B
        • TERT  
        • TERC  
        • RTEL1  
        • PARN  
        • DKC1 
        • TINF2  
        • SFTPC
        • SFTPA2
        • ABCA3
    • Family history of idiopathic pulmonary fibrosis[5]

Less common risk factors

  • Less common risk factors of idiopathic pulmonary fibrosis include the following:


  1. Baumgartner KB, Samet JM, Stidley CA, Colby TV, Waldron JA (1997). "Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis". Am J Respir Crit Care Med. 155 (1): 242–8. doi:10.1164/ajrccm.155.1.9001319. PMID 9001319.
  2. Wolters PJ, Blackwell TS, Eickelberg O, Loyd JE, Kaminski N, Jenkins G; et al. (2018). "Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?". Lancet Respir Med. 6 (2): 154–160. doi:10.1016/S2213-2600(18)30007-9. PMID 29413083.
  3. Armanios, Mary Y.; Chen, Julian J.-L.; Cogan, Joy D.; Alder, Jonathan K.; Ingersoll, Roxann G.; Markin, Cheryl; Lawson, William E.; Xie, Mingyi; Vulto, Irma; Phillips, John A.; Lansdorp, Peter M.; Greider, Carol W.; Loyd, James E. (2007). "Telomerase Mutations in Families with Idiopathic Pulmonary Fibrosis". New England Journal of Medicine. 356 (13): 1317–1326. doi:10.1056/NEJMoa066157. ISSN 0028-4793.
  4. Noth, Imre; Zhang, Yingze; Ma, Shwu-Fan; Flores, Carlos; Barber, Mathew; Huang, Yong; Broderick, Steven M; Wade, Michael S; Hysi, Pirro; Scuirba, Joseph; Richards, Thomas J; Juan-Guardela, Brenda M; Vij, Rekha; Han, MeiLan K; Martinez, Fernando J; Kossen, Karl; Seiwert, Scott D; Christie, Jason D; Nicolae, Dan; Kaminski, Naftali; Garcia, Joe GN (2013). "Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study". The Lancet Respiratory Medicine. 1 (4): 309–317. doi:10.1016/S2213-2600(13)70045-6. ISSN 2213-2600.
  5. Steele MP, Speer MC, Loyd JE, Brown KK, Herron A, Slifer SH; et al. (2005). "Clinical and pathologic features of familial interstitial pneumonia". Am J Respir Crit Care Med. 172 (9): 1146–52. doi:10.1164/rccm.200408-1104OC. PMC 2718398. PMID 16109978.
  6. Tobin RW, Pope CE, Pellegrini CA, Emond MJ, Sillery J, Raghu G (1998). "Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis". Am J Respir Crit Care Med. 158 (6): 1804–8. doi:10.1164/ajrccm.158.6.9804105. PMID 9847271.
  7. Lawson, William E.; Crossno, Peter F.; Polosukhin, Vasiliy V.; Roldan, Juan; Cheng, Dong-Sheng; Lane, Kirk B.; Blackwell, Thomas R.; Xu, Carol; Markin, Cheryl; Ware, Lorraine B.; Miller, Geraldine G.; Loyd, James E.; Blackwell, Timothy S. (2008). "Endoplasmic reticulum stress in alveolar epithelial cells is prominent in IPF: association with altered surfactant protein processing and herpesvirus infection". American Journal of Physiology-Lung Cellular and Molecular Physiology. 294 (6): L1119–L1126. doi:10.1152/ajplung.00382.2007. ISSN 1040-0605.

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