Clinical FeaturesRespiratory

Idiopathic Pulmonary Fibrosis: An Update

Written by Dr Padraig Ridge; MRCPI – Respiratory Specialist Registrar, Galway University Hospital and Dr Sinead Walsh; MRCPI, PhD – Consultant Respiratory and Sleep Physician, Galway University Hospital, Lecturer in Medical Education, NUI Galway

36-38 – HPN January 2022 Digital


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. 2016. Acute exacerbation of idiopathic pulmonary fibrosis. An international working group report. American journal of respiratory and critical care medicine, 194, 265-275.

COTTIN, V., HANSELL, D. M., SVERZELLATI, N., WEYCKER, D., ANTONIOU, K. M., ATWOOD, M., OSTER, G., KIRCHGAESSLER, K.-U., COLLARD, H. R. & WELLS, A. U. 2017. Effect of emphysema extent on serial lung function in patients with idiopathic pulmonary fibrosis. American journal of respiratory and critical care medicine, 196, 1162-1171.

FLAHERTY, K. R., FELL, C. D., HUGGINS, J. T., NUNES, H., SUSSMAN, R., VALENZUELA, C., PETZINGER, U., STAUFFER, J. L., GILBERG, F. & BENGUS, M. 2018. Safety of nintedanib added to pirfenidone treatment for idiopathic pulmonary fibrosis. European Respiratory Journal, 52.

FLAHERTY, K. R., WELLS, A. U., COTTIN, V., DEVARAJ, A., WALSH, S. L., INOUE, Y., RICHELDI, L., KOLB, M., TETZLAFF, K. & STOWASSER, S. 2019. Nintedanib in progressive fibrosing interstitial lung diseases. New England Journal of Medicine, 381, 1718-1727.

GOMES-NETO, M., SILVA, C. M., EZEQUIEL, D., CONCEIÇÃO, C. S., SAQUETTO, M. & MACHADO, A. S. 2018. Impact of pulmonary rehabilitation on exercise tolerance and quality of life in patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Journal of cardiopulmonary rehabilitation and prevention, 38, 273-278.

HEWSON, T., MCKEEVER, T. M., GIBSON, J. E., NAVARATNAM, V., HUBBARD, R. B. & HUTCHINSON, J. P. 2018. Timing of onset of symptoms in people with idiopathic pulmonary fibrosis. Thorax, 73, 683-685. KATO, E., TAKAYANAGI, N., TAKAKU, Y., KAGIYAMA, N., KANAUCHI, T., ISHIGURO, T. & SUGITA, Y. 2018. Incidence and predictive factors of lung cancer in patients with idiopathic pulmonary fibrosis. ERJ open research, 4.

KING JR, 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. 2014. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. New England Journal of Medicine, 370, 2083-2092.

KREUTER, M., WUYTS, W., RENZONI, E., KOSCHEL, D., MAHER, T. M., KOLB, M., WEYCKER, D., SPAGNOLO, P., KIRCHGAESSLER, K.-U. & HERTH, F. J. 2016. Antacid therapy and disease outcomes in idiopathic pulmonary fibrosis: a pooled analysis. The Lancet Respiratory Medicine, 4, 381-389.

LAMAS, D. J., KAWUT, S. M., BAGIELLA, E., PHILIP, N., ARCASOY, S. M. & LEDERER, D. J. 2011. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. American journal of respiratory and critical care medicine, 184, 842-847.

LATSI, P. I., DU BOIS, R. M., NICHOLSON, A. G., COLBY, T. V., BISIRTZOGLOU, D., NIKOLAKOPOULOU, A., VEERARAGHAVAN, S., HANSELL, D. M. & WELLS, A. U. 2003. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. American journal of respiratory and critical care medicine, 168, 531-537.

LEDERER, D. J. & MARTINEZ, F. J. 2018. Idiopathic pulmonary fibrosis. New England Journal of Medicine, 378, 1811-1823.

MARGARITOPOULOS, G. A., ANTONIOU, K. M. & WELLS, A. U. 2017. Comorbidities in interstitial lung diseases. European Respiratory Review, 26.

MARTINEZ, F. J., COLLARD, H. R., PARDO, A., RAGHU, G., RICHELDI, L., SELMAN, M., SWIGRIS, J. J., TANIGUCHI, H. & WELLS, A. U. 2017. Idiopathic pulmonary fibrosis. Nature Reviews Disease Primers, 3, 1-19.

NAKAMURA, Y. & SUDA, T. 2015. Idiopathic pulmonary fibrosis: Diagnosis and clinical manifestations. Clinical Medicine Insights: Circulatory, Respiratory and Pulmonary Medicine, 9, CCRPM. S39897. OLSON, A. L., GIFFORD, A. H., INASE, N., PÉREZ, E. R. F. & SUDA, T. 2018. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. European Respiratory Review, 27.

PARDO, A. & SELMAN, M. 2021. The interplay of the genetic architecture, aging, and environmental factors in the pathogenesis of idiopathic pulmonary fibrosis. American Journal of Respiratory Cell and Molecular Biology, 64, 163-172. RAGHU, G., FREUDENBERGER, T., YANG, S., CURTIS, J., SPADA, C., HAYES, J., SILLERY, J., POPE, C. N. & PELLEGRINI, C. 2006. High prevalence of abnormal acid gastro-oesophageal reflux in IPF. European Respiratory Journal, 27, 136-142.

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. 2018. Diagnosis of IPF.  An official ATS/ERS/JRS/ALAT clinical practice guideline. American journal of respiratory and critical care medicine, 198, e44-e68.

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. 2014. Efficacy and safety of nintedanib in IPF. New England Journal of Medicine, 370, 2071-2082.

RUSH, B., WISKAR, K., BERGER, L. & GRIESDALE, D. 2016. The use of mechanical ventilation in patients with IPF in the United States: a nationwide retrospective cohort analysis. Respiratory medicine, 111, 72-76. WALSH, S. M. & O’REGAN, A. W. 2018. Diffuse Parenchymal Lung Diseases in the Elderly. Current Geriatrics Reports, 7, 174-180.

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