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Closing the GAP in Idiopathic Pulmonary Fibrosis
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Humayun Anjum, MD, FCCP

Click to view more from Pulmonology Data Trends 2024. 

References

  1. 5 things you should know about IPF. American Lung Association. April 12, 2023. Accessed June 21, 2024. https://www.lung.org/blog/idiopathic-pulmonary-fibrosis-things-to-know 

  1. Raghu G, Chen SY, Yeh WS, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med. 2014;2(7):566-572. doi:10.1016/S2213-2600(14)70101-8 

  1. Morrow T. Improving outcomes and managing costs in idiopathic pulmonary fibrosis. Am J Manag Care. 2019;25(11 suppl):S204-S209. PMID: 31419090 

  1. Man RK, Gogikar A, Nanda A, et al. A comparison of the effectiveness of nintedanib and pirfenidone in treating idiopathic pulmonary fibrosis: a systematic review. Cureus. 2024;16(2):e54268. doi:10.7759/cureus.54268 

  1. Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012;156(10):684-691. doi:10.7326/0003-4819-156-10-201205150-00004 

  1. 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 

  1. Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. An International Working Group report. Am J Respir Crit Care Med. 2016;194(3):265-275. doi:10.1164/rccm.201604-0801CI 

  1. Abuserewa ST, Duff R, Becker G. Treatment of idiopathic pulmonary fibrosis. Cureus. 2021;13(5):e15360. doi:10.7759/cureus.15360 

  1. Lee JH, Jang JH, Jang HJ, et al. New prognostic scoring system for mortality in idiopathic pulmonary fibrosis by modifying the gender, age, and physiology model with desaturation during the six-minute walk test. Front Med (Lausanne). 2023;10:1052129. doi:10.3389/fmed.2023.1052129 

  1. Chandel A, Pastre J, Valery S, King CS, Nathan SD. Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis. Thorax. 2023;78(4):368-375. doi:10.1136/thoraxjnl-2021-218440 

  1. Chandel A, King CS, Ignacio RV, et al. External validation and longitudinal application of the DO-GAP index to individualise survival prediction in idiopathic pulmonary fibrosis. ERJ Open Res. 2023;9(3):00124-2023. doi:10.1183/23120541.00124-2023 

  1. Suzuki Y, Mori K, Aono Y, et al. Combined assessment of the GAP index and body mass index at antifibrotic therapy initiation for prognosis of idiopathic pulmonary fibrosis. Sci Rep. 2021;11(1):18579. doi:10.1038/s41598-021-98161-y 

  1. Lacedonia D, De Pace CC, Rea G, et al. Machine learning and BMI improve the prognostic value of GAP index in treated IPF patients. Bioengineering (Basel). 2023;10(2):251. doi:10.3390/bioengineering10020251 

  1. Fujii H, Hara Y, Saigusa Y, et al. ILD-GAP combined with the Charlson Comorbidity Index score (ILD-GAPC) as a prognostic prediction model in patients with interstitial lung disease. Can Respir J. 2023;2023:5088207. doi:10.1155/2023/5088207 

  1. Ley B, Bradford WZ, Weycker D, Vittinghoff E, du Bois RM, Collard HR. Unified baseline and longitudinal mortality prediction in idiopathic pulmonary fibrosis. Eur Respir J. 2015;45(5):1374-1381. doi:10.1183/09031936.00146314 

  1. Kreuter M, Lee JS, Tzouvelekis A, et al. Monocyte count as a prognostic biomarker in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2021;204(1):74-81. doi:10.1164/rccm.202003-0669OC 

  1. Kreuter M, Lee JS, Tzouvelekis A, et al. A modified blood cell GAP (cGAP) to prognosticate outcomes in IPF. Poster presented at: European Respiratory Society International Congress; September 4-6, 2022. https://medically.gene.com/global/en/unrestricted/respiratory/ERS-2022/ers-2022-poster-kreuter-a-modified-blood-cell-gap.html 

  1. Nishikiori H, Chiba H, Lee SH, et al. A modified GAP model for East-Asian populations with idiopathic pulmonary fibrosis. Respir Investig. 2020;58(5):395-402. doi:10.1016/j.resinv.2020.04.001  

Author and Disclosure Information

Humayun Anjum, MD, FCCP
Medical Director, Department of Pulmonary and Critical Care Services
Baylor Scott & White Medical Center
Grapevine, TX

Dr. Anjum has disclosed no relevant financial relationships.

Publications
MDedge Internal Medicine
CHEST Physician
Topics
Pulmonology
Author(s)
Humayun Anjum, MD, FCCP
Author(s)
Humayun Anjum, MD, FCCP
Author and Disclosure Information

Humayun Anjum, MD, FCCP
Medical Director, Department of Pulmonary and Critical Care Services
Baylor Scott & White Medical Center
Grapevine, TX

Dr. Anjum has disclosed no relevant financial relationships.

Author and Disclosure Information

Humayun Anjum, MD, FCCP
Medical Director, Department of Pulmonary and Critical Care Services
Baylor Scott & White Medical Center
Grapevine, TX

Dr. Anjum has disclosed no relevant financial relationships.

Click to view more from Pulmonology Data Trends 2024. 

Click to view more from Pulmonology Data Trends 2024. 

References

  1. 5 things you should know about IPF. American Lung Association. April 12, 2023. Accessed June 21, 2024. https://www.lung.org/blog/idiopathic-pulmonary-fibrosis-things-to-know 

  1. Raghu G, Chen SY, Yeh WS, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med. 2014;2(7):566-572. doi:10.1016/S2213-2600(14)70101-8 

  1. Morrow T. Improving outcomes and managing costs in idiopathic pulmonary fibrosis. Am J Manag Care. 2019;25(11 suppl):S204-S209. PMID: 31419090 

  1. Man RK, Gogikar A, Nanda A, et al. A comparison of the effectiveness of nintedanib and pirfenidone in treating idiopathic pulmonary fibrosis: a systematic review. Cureus. 2024;16(2):e54268. doi:10.7759/cureus.54268 

  1. Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012;156(10):684-691. doi:10.7326/0003-4819-156-10-201205150-00004 

  1. 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 

  1. Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. An International Working Group report. Am J Respir Crit Care Med. 2016;194(3):265-275. doi:10.1164/rccm.201604-0801CI 

  1. Abuserewa ST, Duff R, Becker G. Treatment of idiopathic pulmonary fibrosis. Cureus. 2021;13(5):e15360. doi:10.7759/cureus.15360 

  1. Lee JH, Jang JH, Jang HJ, et al. New prognostic scoring system for mortality in idiopathic pulmonary fibrosis by modifying the gender, age, and physiology model with desaturation during the six-minute walk test. Front Med (Lausanne). 2023;10:1052129. doi:10.3389/fmed.2023.1052129 

  1. Chandel A, Pastre J, Valery S, King CS, Nathan SD. Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis. Thorax. 2023;78(4):368-375. doi:10.1136/thoraxjnl-2021-218440 

  1. Chandel A, King CS, Ignacio RV, et al. External validation and longitudinal application of the DO-GAP index to individualise survival prediction in idiopathic pulmonary fibrosis. ERJ Open Res. 2023;9(3):00124-2023. doi:10.1183/23120541.00124-2023 

  1. Suzuki Y, Mori K, Aono Y, et al. Combined assessment of the GAP index and body mass index at antifibrotic therapy initiation for prognosis of idiopathic pulmonary fibrosis. Sci Rep. 2021;11(1):18579. doi:10.1038/s41598-021-98161-y 

  1. Lacedonia D, De Pace CC, Rea G, et al. Machine learning and BMI improve the prognostic value of GAP index in treated IPF patients. Bioengineering (Basel). 2023;10(2):251. doi:10.3390/bioengineering10020251 

  1. Fujii H, Hara Y, Saigusa Y, et al. ILD-GAP combined with the Charlson Comorbidity Index score (ILD-GAPC) as a prognostic prediction model in patients with interstitial lung disease. Can Respir J. 2023;2023:5088207. doi:10.1155/2023/5088207 

  1. Ley B, Bradford WZ, Weycker D, Vittinghoff E, du Bois RM, Collard HR. Unified baseline and longitudinal mortality prediction in idiopathic pulmonary fibrosis. Eur Respir J. 2015;45(5):1374-1381. doi:10.1183/09031936.00146314 

  1. Kreuter M, Lee JS, Tzouvelekis A, et al. Monocyte count as a prognostic biomarker in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2021;204(1):74-81. doi:10.1164/rccm.202003-0669OC 

  1. Kreuter M, Lee JS, Tzouvelekis A, et al. A modified blood cell GAP (cGAP) to prognosticate outcomes in IPF. Poster presented at: European Respiratory Society International Congress; September 4-6, 2022. https://medically.gene.com/global/en/unrestricted/respiratory/ERS-2022/ers-2022-poster-kreuter-a-modified-blood-cell-gap.html 

  1. Nishikiori H, Chiba H, Lee SH, et al. A modified GAP model for East-Asian populations with idiopathic pulmonary fibrosis. Respir Investig. 2020;58(5):395-402. doi:10.1016/j.resinv.2020.04.001  

References

  1. 5 things you should know about IPF. American Lung Association. April 12, 2023. Accessed June 21, 2024. https://www.lung.org/blog/idiopathic-pulmonary-fibrosis-things-to-know 

  1. Raghu G, Chen SY, Yeh WS, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med. 2014;2(7):566-572. doi:10.1016/S2213-2600(14)70101-8 

  1. Morrow T. Improving outcomes and managing costs in idiopathic pulmonary fibrosis. Am J Manag Care. 2019;25(11 suppl):S204-S209. PMID: 31419090 

  1. Man RK, Gogikar A, Nanda A, et al. A comparison of the effectiveness of nintedanib and pirfenidone in treating idiopathic pulmonary fibrosis: a systematic review. Cureus. 2024;16(2):e54268. doi:10.7759/cureus.54268 

  1. Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012;156(10):684-691. doi:10.7326/0003-4819-156-10-201205150-00004 

  1. 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 

  1. Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. An International Working Group report. Am J Respir Crit Care Med. 2016;194(3):265-275. doi:10.1164/rccm.201604-0801CI 

  1. Abuserewa ST, Duff R, Becker G. Treatment of idiopathic pulmonary fibrosis. Cureus. 2021;13(5):e15360. doi:10.7759/cureus.15360 

  1. Lee JH, Jang JH, Jang HJ, et al. New prognostic scoring system for mortality in idiopathic pulmonary fibrosis by modifying the gender, age, and physiology model with desaturation during the six-minute walk test. Front Med (Lausanne). 2023;10:1052129. doi:10.3389/fmed.2023.1052129 

  1. Chandel A, Pastre J, Valery S, King CS, Nathan SD. Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis. Thorax. 2023;78(4):368-375. doi:10.1136/thoraxjnl-2021-218440 

  1. Chandel A, King CS, Ignacio RV, et al. External validation and longitudinal application of the DO-GAP index to individualise survival prediction in idiopathic pulmonary fibrosis. ERJ Open Res. 2023;9(3):00124-2023. doi:10.1183/23120541.00124-2023 

  1. Suzuki Y, Mori K, Aono Y, et al. Combined assessment of the GAP index and body mass index at antifibrotic therapy initiation for prognosis of idiopathic pulmonary fibrosis. Sci Rep. 2021;11(1):18579. doi:10.1038/s41598-021-98161-y 

  1. Lacedonia D, De Pace CC, Rea G, et al. Machine learning and BMI improve the prognostic value of GAP index in treated IPF patients. Bioengineering (Basel). 2023;10(2):251. doi:10.3390/bioengineering10020251 

  1. Fujii H, Hara Y, Saigusa Y, et al. ILD-GAP combined with the Charlson Comorbidity Index score (ILD-GAPC) as a prognostic prediction model in patients with interstitial lung disease. Can Respir J. 2023;2023:5088207. doi:10.1155/2023/5088207 

  1. Ley B, Bradford WZ, Weycker D, Vittinghoff E, du Bois RM, Collard HR. Unified baseline and longitudinal mortality prediction in idiopathic pulmonary fibrosis. Eur Respir J. 2015;45(5):1374-1381. doi:10.1183/09031936.00146314 

  1. Kreuter M, Lee JS, Tzouvelekis A, et al. Monocyte count as a prognostic biomarker in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2021;204(1):74-81. doi:10.1164/rccm.202003-0669OC 

  1. Kreuter M, Lee JS, Tzouvelekis A, et al. A modified blood cell GAP (cGAP) to prognosticate outcomes in IPF. Poster presented at: European Respiratory Society International Congress; September 4-6, 2022. https://medically.gene.com/global/en/unrestricted/respiratory/ERS-2022/ers-2022-poster-kreuter-a-modified-blood-cell-gap.html 

  1. Nishikiori H, Chiba H, Lee SH, et al. A modified GAP model for East-Asian populations with idiopathic pulmonary fibrosis. Respir Investig. 2020;58(5):395-402. doi:10.1016/j.resinv.2020.04.001  

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Closing the GAP in Idiopathic Pulmonary Fibrosis
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Closing the GAP in Idiopathic Pulmonary Fibrosis
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IPF, the most prevalent ILD and one of unknown etiology, affects up to 207,000 Americans and up to 58,000 new patients each year.1 Prognosis is poor; median survival estimates have ranged between 2 and 5 years for the last decade.2,3 Although IPF is not curable, initiating a treatment plan as early as possible is critical to managing symptoms and slowing disease progression.4

Introduced in 2012, the GAP (gender, age, physiology) prognostic model offers clinicians a framework for assessing mortality risk, with the goal of improving IPF outcomes.5 The GAP model uses a standardized approach to staging patients while also aiding clinicians in tailoring each patient’s treatment approach.5-8 The “physiology” component evaluates forced vital capacity (FVC) to assess lung function and diffusing capacity of the lungs for carbon monoxide (DLCO) to measure gas exchange effciency.

While the integration of FVC and DLCO into the GAP model provided a more comprehensive assessment at the time of its introduction, our understanding of IPF has evolved over the last decade. There has been a recent surge in proposed modications to the original GAP model. Studies have examined the integration of additional criteria, such as comorbidities, body mass index (BMI), exercise capacity, and other factors, into the GAP model to help to improve predictive precision.9-15 The incorporation of additional parameters and biological markers offers promising prospects for more accurate prognostications and personalized treatment strategies. Although these proposed enhancements to the GAP model require further validation, their potential to refine treatment personalization makes them worthy of careful consideration.

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