Idiopathic pulmonary fibrosis
Last updated 18 Aug 2020
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Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. This condition causes scar tissue (fibrosis) to build up in the lungs, which makes the lungs unable to transport oxygen into the bloodstream effectively. The disease usually affects people between the ages of 50 and 70. Idiopathic pulmonary fibrosis belongs to a group of conditions called interstitial lung diseases (also known as ILD), which describes lung diseases that involve inflammation or scarring in the lung.
Description
Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. This condition causes scar tissue (fibrosis) to build up in the lungs, which makes the lungs unable to transport oxygen into the bloodstream effectively. The disease usually affects people between the ages of 50 and 70. Idiopathic pulmonary fibrosis belongs to a group of conditions called interstitial lung diseases (also known as ILD), which describes lung diseases that involve inflammation or scarring in the lung.
The most common signs and symptoms of idiopathic pulmonary fibrosis are shortness of breath and a persistent dry, hacking cough. Many affected individuals also experience a loss of appetite and gradual weight loss. Some people with idiopathic pulmonary fibrosis develop widened and rounded tips of the fingers and toes (clubbing) resulting from a shortage of oxygen. These features are relatively nonspecific; not everyone with these health problems has idiopathic pulmonary fibrosis. Other respiratory diseases, some of which are less serious, can cause similar signs and symptoms.
In people with idiopathic pulmonary fibrosis, scarring of the lungs increases over time until the lungs can no longer provide enough oxygen to the body's organs and tissues. Some people with idiopathic pulmonary fibrosis develop other serious lung conditions, including lung cancer, blood clots in the lungs (pulmonary emboli), pneumonia, or high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Most affected individuals survive 3 to 5 years after their diagnosis. However, the course of the disease is highly variable; some affected people become seriously ill within a few months, while others may live with the disease for a decade or longer.
In most cases, idiopathic pulmonary fibrosis occurs in only one person in a family. These cases are described as sporadic. However, a small percentage of people with this disease have at least one other affected family member. When idiopathic pulmonary fibrosis occurs in multiple members of the same family, it is known as familial pulmonary fibrosis.
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Other names
Cryptogenic fibrosing alveolitis
Idiopathic fibrosing alveolitis, chronic form
IPF
Usual interstitial pneumonia
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Further reading and references
- Idiopathic pulmonary fibrosis; MedlinePlus, National Library of Medicine.
- Allen RJ, Guillen-Guio B, Oldham JM, Ma SF, Dressen A, Paynton ML, Kraven LM, Obeidat M, Li X, Ng M, Braybrooke R, Molina-Molina M, Hobbs BD, Putman RK, Sakornsakolpat P, Booth HL, Fahy WA, Hart SP, Hill MR, Hirani N, Hubbard RB, McAnulty RJ, Millar AB, Navaratnam V, Oballa E, Parfrey H, Saini G, Whyte MKB, Zhang Y, Kaminski N, Adegunsoye A, Strek ME, Neighbors M, Sheng XR, Gudmundsson G, Gudnason V, Hatabu H, Lederer DJ, Manichaikul A, Newell JD Jr, O'Connor GT, Ortega VE, Xu H, Fingerlin TE, Bosse Y, Hao K, Joubert P, Nickle DC, Sin DD, Timens W, Furniss D, Morris AP, Zondervan KT, Hall IP, Sayers I, Tobin MD, Maher TM, Cho MH, Hunninghake GM, Schwartz DA, Yaspan BL, Molyneaux PL, Flores C, Noth I, Jenkins RG, Wain LV. Genome-Wide Association Study of Susceptibility to Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2020 Mar 1;201(5):564-574. doi: 10.1164/rccm.201905-1017OC.
- Allen RJ, Porte J, Braybrooke R, Flores C, Fingerlin TE, Oldham JM, Guillen-Guio B, Ma SF, Okamoto T, John AE, Obeidat M, Yang IV, Henry A, Hubbard RB, Navaratnam V, Saini G, Thompson N, Booth HL, Hart SP, Hill MR, Hirani N, Maher TM, McAnulty RJ, Millar AB, Molyneaux PL, Parfrey H, Rassl DM, Whyte MKB, Fahy WA, Marshall RP, Oballa E, Bosse Y, Nickle DC, Sin DD, Timens W, Shrine N, Sayers I, Hall IP, Noth I, Schwartz DA, Tobin MD, Wain LV, Jenkins RG. Genetic variants associated with susceptibility to idiopathic pulmonary fibrosis in people of European ancestry: a genome-wide association study. Lancet Respir Med. 2017 Nov;5(11):869-880. doi: 10.1016/S2213-2600(17)30387-9. Epub 2017 Oct 20.
- American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med. 2000 Feb;161(2 Pt 1):646-64. doi: 10.1164/ajrccm.161.2.ats3-00. No abstract available.
- Arish N, Petukhov D, Wallach-Dayan SB. The Role of Telomerase and Telomeres in Interstitial Lung Diseases: From Molecules to Clinical Implications. Int J Mol Sci. 2019 Jun 19;20(12):2996. doi: 10.3390/ijms20122996.
- Armanios MY, Chen JJ, Cogan JD, Alder JK, Ingersoll RG, Markin C, Lawson WE, Xie M, Vulto I, Phillips JA 3rd, Lansdorp PM, Greider CW, Loyd JE. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med. 2007 Mar 29;356(13):1317-26. doi: 10.1056/NEJMoa066157.
- Borchers AT, Chang C, Keen CL, Gershwin ME. Idiopathic pulmonary fibrosis-an epidemiological and pathological review. Clin Rev Allergy Immunol. 2011 Apr;40(2):117-34. doi: 10.1007/s12016-010-8211-5.
- Garcia CK, Talbert JL. Pulmonary Fibrosis Predisposition Overview. 2005 Jan 21 [updated 2025 Dec 4]. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK1230/
- Harari S, Caminati A. IPF: new insight on pathogenesis and treatment. Allergy. 2010 May;65(5):537-53. doi: 10.1111/j.1398-9995.2009.02305.x. Epub 2010 Feb 1.
- Hobbs BD, Putman RK, Araki T, Nishino M, Gudmundsson G, Gudnason V, Eiriksdottir G, Zilhao Nogueira NR, Dupuis J, Xu H, O'Connor GT, Manichaikul A, Nguyen J, Podolanczuk AJ, Madahar P, Rotter JI, Lederer DJ, Barr RG, Rich SS, Ampleford EJ, Ortega VE, Peters SP, O'Neal WK, Newell JD Jr, Bleecker ER, Meyers DA, Allen RJ, Oldham JM, Ma SF, Noth I, Jenkins RG, Maher TM, Hubbard RB, Wain LV, Fingerlin TE, Schwartz DA, Washko GR, Rosas IO, Silverman EK, Hatabu H, Cho MH, Hunninghake GM. Overlap of Genetic Risk between Interstitial Lung Abnormalities and Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2019 Dec 1;200(11):1402-1413. doi: 10.1164/rccm.201903-0511OC.
- Kolb M, Vasakova M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019 Mar 14;20(1):57. doi: 10.1186/s12931-019-1022-1.
- McLean-Tooke A, Moore I, Lake F. Idiopathic and immune-related pulmonary fibrosis: diagnostic and therapeutic challenges. Clin Transl Immunology. 2019 Nov 5;8(11):e1086. doi: 10.1002/cti2.1086. eCollection 2019.
- Ryu JH, Moua T, Daniels CE, Hartman TE, Yi ES, Utz JP, Limper AH. Idiopathic pulmonary fibrosis: evolving concepts. Mayo Clin Proc. 2014 Aug;89(8):1130-42. doi: 10.1016/j.mayocp.2014.03.016. Epub 2014 May 24.
- Stuart BD, Lee JS, Kozlitina J, Noth I, Devine MS, Glazer CS, Torres F, Kaza V, Girod CE, Jones KD, Elicker BM, Ma SF, Vij R, Collard HR, Wolters PJ, Garcia CK. Effect of telomere length on survival in patients with idiopathic pulmonary fibrosis: an observational cohort study with independent validation. Lancet Respir Med. 2014 Jul;2(7):557-65. doi: 10.1016/S2213-2600(14)70124-9. Epub 2014 Jun 16.
- Tsakiri KD, Cronkhite JT, Kuan PJ, Xing C, Raghu G, Weissler JC, Rosenblatt RL, Shay JW, Garcia CK. Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc Natl Acad Sci U S A. 2007 May 1;104(18):7552-7. doi: 10.1073/pnas.0701009104. Epub 2007 Apr 25.
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