The DOI matches its Crossref record; 65 of the 65 references it cites were found in a registry or an index.
Antifibrotic therapy for idiopathic pulmonary fibrosis: time to treat
Open accessAbstract
Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a dismal prognosis. The average life expectancy of untreated patients with IPF is only 3 to 4 years. Decline in forced vital capacity (FVC) in patients with IPF appears to be almost linear, with patients with well-preserved FVC at baseline experiencing the same rate of decline in FVC as patients with more advanced disease. Two antifibrotic therapies have been approved for the treatment of IPF: nintedanib and pirfenidone. These drugs slow decline in lung function and reduce the risk of acute respiratory deteriorations, which are associated with very high morbidity and mortality. Individual clinical trials have not been powered to show reductions in mortality, but analyses of pooled data from clinical trials, as well as observational studies, suggest that antifibrotic therapies improve life expectancy. Despite this, many individuals with IPF remain untreated. In many cases, this is because the physician perceives that the disease is stable and so does not warrant therapy, or has concerns over the potential side-effects of antifibrotic drugs. There remains a need to educate pulmonologists that IPF is a progressive, irreversible and fatal disease and that prompt treatment is critical to preserving patients' lung function and improving outcomes. Most individuals can tolerate antifibrotic therapy, and dose adjustment has been shown to be effective at reducing side effects without compromising efficacy. In addition to anti-fibrotic therapies, individuals with IPF benefit from a holistic approach to their care that includes symptom management and supportive care tailored to the needs of the individual. An animation illustrating the themes covered in this article will be available at: http://www.usscicomms.com/respiratory/maher/treatment-of-IPF .
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Citations
Cites
Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis
Richeldi, L., du Bois, R. M., Raghu, G., Azuma, A., Brown, K. K., Costabel, U., Cottin, V., Flaherty, K. R., Hansell, D. M., Inoue, Y., et al. (2014) · New England Journal of Medicine · doi:10.1056/nejmoa1402584 · Cited by 4,654 · cited 1×
“The Phase III INPULSIS and ASCEND trials showed that in subjects with mild or moderate FVC impairment at baseline, nintedanib and pirfenidone reduced the rate of decline in FVC by approximately 50% over 1 year of treatment [20, 21].”
MethodDiagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline
Raghu, G., Remy-Jardin, M., Myers, J. L., Richeldi, L., Ryerson, C. J., Lederer, D. J., Behr, J., Cottin, V., Danoff, S. K., Morell, F., et al. (2018) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201807-1255st · Cited by 4,103 · cited 1×
“Background IPF is a progressive and ultimately fatal interstitial lung disease (ILD) characterized by radiologic and/or histopathologic findings of usual interstitial pneumonia [1].”
BackgroundA phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis.
King, T. E., Bradford, W. Z., Castro-Bernardini, S., Fagan, E. A., Glaspole, I., Glassberg, M. K., Gorina, E., Hopkins, P. M., Kardatzke, D., Lancaster, L., et al. (2014) · New England Journal of Medicine · doi:10.1056/nejmoa1402582 · Cited by 3,975
Raghu, G., Rochwerg, B., Zhang, Y., Garcia, C. A. C., Azuma, A., Behr, J., Brozek, J. L., Collard, H. R., Cunningham, W., Homma, S., et al. (2015) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201506-1063st · Cited by 1,916
Prednisone, Azathioprine, and N -Acetylcysteine for Pulmonary Fibrosis
The Idiopathic Pulmonary Fibrosis Clinical Research Network (2012) · New England Journal of Medicine · doi:10.1056/nejmoa1113354 · Cited by 1,621
Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report
Collard, H. R., Ryerson, C. J., Corte, T. J., Jenkins, G., Kondoh, Y., Lederer, D. J., Lee, J. S., Maher, T. M., Wells, A. U., Antoniou, K. M., et al. (2016) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201604-0801ci · Cited by 1,444
Self-citationEfficacy of a Tyrosine Kinase Inhibitor in Idiopathic Pulmonary Fibrosis
Richeldi, L., Costabel, U., Selman, M., Kim, D. S., Hansell, D. M., Nicholson, A. G., Brown, K. K., Flaherty, K. R., Noble, P. W., Raghu, G., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1103690 · Cited by 1,157
Mode of action of nintedanib in the treatment of idiopathic pulmonary fibrosis
Wollin, L., Wex, E., Pautsch, A., Schnapp, G., Hostettler, K. E., Stowasser, S., & Kolb, M. (2015) · European Respiratory Journal · doi:10.1183/09031936.00174914 · Cited by 1,005
Raghu, G., Chen, S.-Y., Yeh, W.-S., Maroni, B., Li, Q., Lee, Y.-C., & Collard, H. R. (2014) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(14)70101-8 · Cited by 716
A Controlled Trial of Sildenafil in Advanced Idiopathic Pulmonary Fibrosis
(2010) · New England Journal of Medicine · doi:10.1056/nejmoa1002110 · Cited by 634
Cited by
Antifibrotic therapy in nonalcoholic steatohepatitis: time for a human-centric approach
Brennan, P. N., Elsharkawy, A. M., Kendall, T. J., Loomba, R., Mann, D. A., & Fallowfield, J. A. (2023) · Nature Reviews Gastroenterology & Hepatology · doi:10.1038/s41575-023-00796-x · Cited by 68
Belhassen, M., Dalon, F., Nolin, M., & Van Ganse, E. (2021) · Respiratory Research · doi:10.1186/s12931-021-01714-y · Cited by 38
Man, R. K., Gogikar, A., Nanda, A., Janga, L. S. N., Sambe, H. G., Yasir, M., & Ramphall, S. (2024) · Cureus · doi:10.7759/cureus.54268 · Cited by 30
Zhang, Y., MacKenzie, B., Koleng, J. J., Maier, E., Warnken, Z. N., & Williams, R. O. (2020) · Molecular Pharmaceutics · doi:10.1021/acs.molpharmaceut.9b01085 · Cited by 27
Blaskovic, S., Donati, Y., Ruchonnet-Metrailler, I., Seredenina, T., Krause, K.-H., Pache, J.-C., Adler, D., Barazzone-Argiroffo, C., & Jaquet, V. (2021) · Antioxidants · doi:10.3390/antiox10111833 · Cited by 11
Metadata
- Authors
- Maher, T. M., & Strek, M. E.
- Venue
- Respiratory Research
- Published
- 2019-09-06
- Volume
- 20
- Issue
- 1
- Pages
- 205-205
- ISSN
- 1465-9921 (ISSN-L)
- 1465-993X
- OpenAlex
- W2972058749
- Cited by
- 265
- References
- 65
- FWCI
- 21.58
- Type
- journal article
- Language
- en
- Open access
- gold
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