The impact of FVC/DLCO ratio on diagnosis of pulmonary hypertension and disease prognosis in idiopathic pulmonary fibrosis
Abstract
Introduction: Idiopathic pulmonary fibrosis is a chronic progressive fibrotic lung disease of unknown etiology that occurs most commonly in older adults. The presence of pulmonary hypertension in Idiopathic pulmonary fibrosis is associated with poor prognosis and mortality. Literature suggests that the forced vital capacity to diffusion capacity of the lung for carbon monoxide ratio has a positive predictive value for the diagnosis of pulmonary hypertension. Therefore, this study aimed to investigate the impact of forced vital capacity to diffusion capacity of the lung for carbon monoxide ratio on the diagnosis of pulmonary hypertension and disease prognosis in Idiopathic pulmonary fibrosis patients.
MATERIAL AND METHOD: Forty-eight patients diagnosed with Idiopathic pulmonary fibrosis were included in the study. Patient records, echocardiographic and spirometric data were retrospectively reviewed.
Results: The average pulmonary arterial pressure was observed to be 32.8 (±9) mmHg, with the second-year follow-up pulmonary arterial pressure at 40.8 (±17.2) mmHg and the fourth-year follow-up pulmonary arterial pressure at 51 (±23.6) mmHg. In those diagnosed as pulmonary hypertension, the forced vital capacity to diffusion capacity of the lung for carbon monoxide ratio was initially 1.54 (±0.72). By the second year, it was 1.61 (±0.45), and by the fourth year, it was 1.87 (±0.8). It was found that the forced vital capacity to diffusion capacity of the lung for carbon monoxide ratio tended to increase when pulmonary artery pressure increased during the follow-up period.
Conclusion: We found that low six- minute walking test distance was an important marker for the diagnosis of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and that the presence of desaturation was also significantly associated with survival in pulmonary hypertension. Although we did not find it statistically significant, we found that both pulmonary arterial pressure and the the forced vital capacity to diffusion capacity of the lung for carbon monoxide ratio increased with progressive disease duration after diagnosis in patients with IPF. We believe that the the forced vital capacity to diffusion capacity of the lung for carbon monoxide ratio is an important marker for early detection of pulmonary hypertension and prognosis in idiopathic pulmonary fibrosis.
Supporting Institution
YOK
Project Number
YOK
Thanks
YPOK
References
- 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: 788-824.
- Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008; 3: 8.
- Martinez FJ, Flaherty K. Pulmonary function testing in idiopathic intersti tial pneumonias. Proc Am Thorac Soc 2006; 3: 315–21.
- Zappala CJ, Latsi PI, Nicholson AG, et al. Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fbrosis. Eur Respir J 2010; 35: 830–6.
- Cano-Jiménez E, Hernández González F, Peloche GB. Comorbidities and complications in ıdiopathic pulmonary fibrosis. Med Sci 2018; 6: 71.
- Collum SD, Amione-Guerra J, Cruz-Solbes AS, et al. Pulmonary hypertension associated with ıdiopathic pulmonary fibrosis: current and future perspectives. Can Respir J 2017; 2017: 1430350.
- Sherner J, Collen J, King CS, Nathan SD. Pulmonary hypertension in idiopathic pulmonary fibrosis: epidemiology, diagnosis, and therapeutic implications. Curr Respir Care Rep 2012; 1: 233-42.
- Behr J, Ryu JH. Pulmonary hypertension in interstitial lung disease. Eur Respir J 2008; 31: 1357-67.
- Shorr AF, Wainright JL, Cors CS, et al. Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant. Eur Respir J 2007; 30: 715-21.
- Kimura, Taniguchi H, Kondoh Y, et al. Pulmonary hypertension as a prognostic indicator at the initial evaluation in idiopathic pulmonary fibrosis. Respiration 2013; 85: 456-63.
- Nathan SD, King CS. Treatment of pulmonary hypertension in idiopathic pulmonary fibrosis: shortfall in efficacy or trial design? Drug Des Devel Ther 2014; 8: 875-85.
- Zisman DA, Ross DJ, Belperio JA, et al. Prediction of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 2007; 101: 2153-9.
- Steen VD, Graham G, Conte C, Owens G, Medsger TA Jr. Isolated diffusing capacity reduction in systemic sclerosis. Arthritis Rheum 1992; 35: 765-70.
- Öztürk A, Kayacan O. Rising stars of DPLD survival: FVC and exercise desaturation. Turk J Med Sci 2018; 48: 826-32.
- Yock PG, Popp RL. Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation 1984; 70: 657–62.
- European RS, American Thoracic Society. ATS/ ERS Statementon respiratory muscle testing. Am J Respir Crit Care Med 2002;166: 518-624.
- Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med 2012; 156: 684–95.
- Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan a parallel, randomized trial. Annals of Internal Medicine 2013; 158: 641-9.
- Teramachi R, Taniguchi H, Kondoh Y, et al. Progression of mean pulmonary arterial pressure in idiopathic pulmonary fibrosis with mild to moderate restriction. Respirology 2017; 22: 986-90.
- Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006; 129: 746-52.
- du Bois R, Albera C, Bradford W, et al. Predictors of mortality in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009; 179: A1114.
- Hanson D, Winterbauer RH, Kirtland SH, Wu R. Changes in pulmonary function test results after 1 year therapy as predictors of survival in patients with idiopathic pulmonary fibrosis. Chest 1995; 108: 305-10.
- Caminati A, Bianchi A, Cassandro R, Mirenda MR, Harari S. Walking distance on 6-MWT is a prognostic factor in idiopathic pulmonary fibrosis. Respir Med 2009; 103: 117-23.
- Shapiro S. Management of pulmonary hypertension resulting from interstitial lung disease. Curr Opin Pulm Med 2003; 9: 426-30.
- Strange C, Highland KB. Pulmonary hypertension in interstitial lung disease. Curr Opin Pulm Med 2005; 11: 452-5.
- Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 183: 431-40.
- Salisbury ML, Xia M, Zhou Y, et al. Idiopathic pulmonary fibrosis: gender-age-physiology ındex stage for predicting future lung function decline. Chest 2016; 149: 491-8.
- Song H, Sun D, Ban C, et al. Independent clinical factors relevant to prognosis of patients with ıdiopathic pulmonary fibrosis. Med Sci Monit 2019; 25: 4193–201.
- Olson AL, Swigris JJ, Lezotte DC, et al. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med 2007; 176: 277–84.
Abstract
Project Number
YOK
References
- 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: 788-824.
- Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008; 3: 8.
- Martinez FJ, Flaherty K. Pulmonary function testing in idiopathic intersti tial pneumonias. Proc Am Thorac Soc 2006; 3: 315–21.
- Zappala CJ, Latsi PI, Nicholson AG, et al. Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fbrosis. Eur Respir J 2010; 35: 830–6.
- Cano-Jiménez E, Hernández González F, Peloche GB. Comorbidities and complications in ıdiopathic pulmonary fibrosis. Med Sci 2018; 6: 71.
- Collum SD, Amione-Guerra J, Cruz-Solbes AS, et al. Pulmonary hypertension associated with ıdiopathic pulmonary fibrosis: current and future perspectives. Can Respir J 2017; 2017: 1430350.
- Sherner J, Collen J, King CS, Nathan SD. Pulmonary hypertension in idiopathic pulmonary fibrosis: epidemiology, diagnosis, and therapeutic implications. Curr Respir Care Rep 2012; 1: 233-42.
- Behr J, Ryu JH. Pulmonary hypertension in interstitial lung disease. Eur Respir J 2008; 31: 1357-67.
- Shorr AF, Wainright JL, Cors CS, et al. Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant. Eur Respir J 2007; 30: 715-21.
- Kimura, Taniguchi H, Kondoh Y, et al. Pulmonary hypertension as a prognostic indicator at the initial evaluation in idiopathic pulmonary fibrosis. Respiration 2013; 85: 456-63.
- Nathan SD, King CS. Treatment of pulmonary hypertension in idiopathic pulmonary fibrosis: shortfall in efficacy or trial design? Drug Des Devel Ther 2014; 8: 875-85.
- Zisman DA, Ross DJ, Belperio JA, et al. Prediction of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 2007; 101: 2153-9.
- Steen VD, Graham G, Conte C, Owens G, Medsger TA Jr. Isolated diffusing capacity reduction in systemic sclerosis. Arthritis Rheum 1992; 35: 765-70.
- Öztürk A, Kayacan O. Rising stars of DPLD survival: FVC and exercise desaturation. Turk J Med Sci 2018; 48: 826-32.
- Yock PG, Popp RL. Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation 1984; 70: 657–62.
- European RS, American Thoracic Society. ATS/ ERS Statementon respiratory muscle testing. Am J Respir Crit Care Med 2002;166: 518-624.
- Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med 2012; 156: 684–95.
- Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan a parallel, randomized trial. Annals of Internal Medicine 2013; 158: 641-9.
- Teramachi R, Taniguchi H, Kondoh Y, et al. Progression of mean pulmonary arterial pressure in idiopathic pulmonary fibrosis with mild to moderate restriction. Respirology 2017; 22: 986-90.
- Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006; 129: 746-52.
- du Bois R, Albera C, Bradford W, et al. Predictors of mortality in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009; 179: A1114.
- Hanson D, Winterbauer RH, Kirtland SH, Wu R. Changes in pulmonary function test results after 1 year therapy as predictors of survival in patients with idiopathic pulmonary fibrosis. Chest 1995; 108: 305-10.
- Caminati A, Bianchi A, Cassandro R, Mirenda MR, Harari S. Walking distance on 6-MWT is a prognostic factor in idiopathic pulmonary fibrosis. Respir Med 2009; 103: 117-23.
- Shapiro S. Management of pulmonary hypertension resulting from interstitial lung disease. Curr Opin Pulm Med 2003; 9: 426-30.
- Strange C, Highland KB. Pulmonary hypertension in interstitial lung disease. Curr Opin Pulm Med 2005; 11: 452-5.
- Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 183: 431-40.
- Salisbury ML, Xia M, Zhou Y, et al. Idiopathic pulmonary fibrosis: gender-age-physiology ındex stage for predicting future lung function decline. Chest 2016; 149: 491-8.
- Song H, Sun D, Ban C, et al. Independent clinical factors relevant to prognosis of patients with ıdiopathic pulmonary fibrosis. Med Sci Monit 2019; 25: 4193–201.
- Olson AL, Swigris JJ, Lezotte DC, et al. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med 2007; 176: 277–84.
Details
| Primary Language | English |
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| Subjects | Health Care Administration |
| Journal Section | Original Article |
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| Project Number | YOK |
| Publication Date | October 25, 2022 |
| Published in Issue | Year 2022 Volume: 5 Issue: 6 |
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