Risk Factors of IPF
RISK FACTORS
MOST COMMON RISK FACTORS FOR IPF
IPF is a chronic fibrosing lung disease of unknown cause(s).1,2 However, many factors have been associated with increased risk of developing IPF.1,2
The most common risk factors for IPF are old age, history of smoking, and male gender
- 65+:3-5 During a recent study, the mean age at diagnosis of IPF was 63.6
- Males have a higher prevalence of IPF than females.3,5,7,8
- Smoking history shows a significant association with risk of developing IPF.5
POTENTIAL RISK FACTORS FOR IDIOPATHIC PULMONARY FIBROSIS (IPF):
- Age
- Gender
- Smoking
- Genetics
Age
- Increased age is one of the most common risk factors for IPF2
- The patient's age at presentation is an important consideration2,9
- IPF typically occurs in individuals between 60 and 70 years of age1,10
- IPF is only rarely found in patients younger than 501
- In older people, the most common type of interstitial lung disease (ILD) is IPF9
- Older age is highly associated with risk of IPF9
- Age ≥70: 95% positive predictive value (PPV) for IPF
- Age ≥75: ~100% PPV for IPF
Gender
- Male gender is a common risk factor for IPF3,5,7,8
- The prevalence of interstitial lung disease is 20% higher in men than in women8
- In a study of 920 patients with IPF, 62% were men7
Smoking
- A history of smoking is a common risk factor for IPF1,11
- There is a strong association between smoking and IPF, especially for those with a greater than 20 pack-years history11
- Exposure to tobacco smoke is a risk factor for both sporadic and familial IPF12,13
- Additionally, tobacco history may be associated with more rapid disease progression14
Genetics
- Several environmental and genetic risk factors have been described despite its unknown etiology1,2
- Genetic factors have been linked to both sporadic and familial IPF1,2,15
- It is estimated that 5% of cases of IPF are familial2
- A family history of IPF is a strong risk factor, increasing the odds up to 6-fold16
- Familial and sporadic IPF are clinically, radiologically, and histologically identical2
It is hypothesized that these environmental and genetic risk factors contribute to epithelial cell injury and apoptosis during disease progression.17,18
REFERENCES
- Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68. doi: 10.1164/rccm.201807-1255ST.
- Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824. doi: 10.1164/rccm.2009-040GL.
- King TE, Tooze JA, Schwarz MI, et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med. 2001;164(7):1171-81. doi: 10.1164/ajrccm.164.7.2003140.
- Kim DS, Collard HR, King TE. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006;3(4):285-292. doi: 10.1513/pats.200601-005TK.
- Douglas W, Ryu JH, Schroeder DR, et al. Idiopathic pulmonary fibrosis: Impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med. 2000; 161(4 Pt 1):1172-1178. doi: 10.1164/ajrccm.161.4.9907002.
- van Moorsel CHM, van Oosterhout MFM, Barlo NP, et al. Surfactant protein C mutations are the basis of a significant portion of adult familial pulmonary fibrosis in a dutch cohort. Am J Respir Crit Care Med. 2010;182(11):1419-1425. doi: 10.1164/rccm.200906-0953OC.
- Gribbin J, Hubbard RB, Le Juene I, et al. Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. J Thorax. 2006;61:980-985. doi: 10.1136/thx.2006.062836.
- Coultas DB, Zumwalt RE, Black WC, et al. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med. 1994;150(4):967-972. doi: 10.1164/ajrccm.150.4.7921471.
- Fell CD, Martinez FJ, Liu LX, et al. Clinical predictors of a diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2010;181(8):832-837. doi: 10.1164/rccm.200906-0959OC.
- Castriotta RJ, Eldadah BA, Foster WM, et al. Workshop on idiopathic pulmonary fibrosis in older adults. Chest. 2010;138(3):693-703. doi: 10.1378/chest.09-3006.
- Baumgartner KB, Samet JM, Stidley CA, et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am I Respir Crit Care Med. 1997;155(1):242-248. doi: 10.1164/ajrccm.155.1.9001319.
- Steele MP, Speer MC, Loyd JL, et al. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 2005;172(9):1146-1152. doi: 10.1164/rccm.200408-1104OC.
- Taskar VS, Coultas DB. Is idiopathic pulmonary fibrosis an environmental disease? Proc Am Thorac Soc. 2006;3(4):293-298. doi: 10.1513/pats.200512-131TK.
- Selman M, Carrillo G, Estrada A, et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One. 2007;2(5):e482. doi: 10.1371/journal.pone.0000482.
- Fernandez BA, Fox G, Bhatia R, et al. A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features. Respir Res. 2012;13(1):64. doi: 10.1186/1465-9921-13-64.
- Garcia-Sancho C, Buendía-Roldán I, Fernández-Plata MR, et al. Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis. Respir Med. 2011;105(12):1902-1907. doi: 10.1016/j.rmed.2011.08.022.
- Fernandez BA, Fox G, Bhatia R, et al. A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features. Respir Res. 2012;13(1):64. doi: 10.1186/1465-9921-13-64.
- Camelo A, Dunmore R, Sleeman MA, et al. The epithelium in idiopathic pulmonary fibrosis: breaking the barrier. Front Pharmacol. 2014:4:173. doi: 10.3389/fphar.2013.00173.
