間質性肺疾病合并肺動脈高壓的研究進展_Chinese Journal of Respiratory and Critical Care Medicine

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郝亞楠 ¹ ,  成孟瑜 ^1,2

1. ;
2. ;

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成孟瑜, Email: chengmengyu@sxbqeh.com.cn

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DOI：

10.7507/1671-6205.202506061

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Citation： 郝亞楠, 成孟瑜. 間質性肺疾病合并肺動脈高壓的研究進展. Chinese Journal of Respiratory and Critical Care Medicine, 2026, 25(3): 210-216. doi: 10.7507/1671-6205.202506061 Copy

1.	Zhao N, Chen J, Zhang M, et al. PAH-specific therapy for pulmonary hypertension and interstitial lung disease: a systemic review and meta-analysis. Front Cardiovasc Med, 2022, 9: 992879.
2.	Wells AU, Brown KK, Flaherty KR, et al. What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J, 2018, 51(5): 1800692.
3.	Ruaro B, Baratella E, Confalonieri P, et al. High-resolution computed tomography: Lights and shadows in improving care for SSc-ILD patients. Diagnostics (Basel), 2021, 11(11): 1960.
4.	Nathan SD, Barbera JA, Gaine SP, et al. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J, 2019, 53(1): 1801914.
5.	King CS, Shlobin OA. The trouble with group 3 pulmonary hypertension in interstitial lung disease: Dilemmas in diagnosis and the conundrum of treatment. Chest, 2020, 158(4): 1651-1664.
6.	Waxman AB, Elia D, Adir Y, et al. Recent advances in the management of pulmonary hypertension with interstitial lung disease. Eur Respir Rev, 2022, 31(165): 210220.
7.	Sd C, J AG, As CS, et al. Pulmonary hypertension associated with idiopathic pulmonary fibrosis: Current and future perspectives. Can Respir J, 2017, 2017: 1430350.
8.	Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease. N Engl J Med, 2021, 384(4): 325-334.
9.	Nathan SD, Behr J, Cottin V, et al. Study design and rationale for the TETON phase 3, randomised, controlled clinical trials of inhaled treprostinil in the treatment of idiopathic pulmonary fibrosis. BMJ Open Respir Res, 2022, 9(1): e001310.
10.	Johnson S, Sommer N, Cox-Flaherty K, et al. Pulmonary hypertension: a contemporary review. Am J Respir Crit Care Med, 2023, 208(5): 528-548.
11.	Mocumbi A, Humbert M, Saxena A, et al. Pulmonary hypertension. Nat Rev Dis Primers, 2024, 10(1): 1.
12.	Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J, 2019, 53(1): 1801913.
13.	Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J, 2023, 61(1): 2200879.
14.	Wijsenbeek M, Suzuki A, Maher TM. Interstitial lung diseases. Lancet, 2022, 400(10354): 769-786.
15.	Braganza M, Shaw J, Solverson K, et al. A prospective evaluation of the diagnostic accuracy of the physical examination for pulmonary hypertension. Chest, 2019, 155(5): 982-990.
16.	徐婉婷, 江曉琴, 朱濤. 肺動脈高壓的治療進展. 中國呼吸與危重監護雜志, 2024, 23(4): 287-292.
17.	Olsson KM, Corte TJ, Kamp JC, et al. Pulmonary hypertension associated with lung disease: new insights into pathomechanisms, diagnosis, and management. Lancet Respir Med, 2023, 11(9): 820-835.
18.	Shlobin OA, Adir Y, Barbera JA, et al. Pulmonary hypertension associated with lung diseases. Eur Respir J, 2024, 64(4): 2401200.
19.	Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev, 2018, 27(150): 180076.
20.	樊銘薇, 蔣天賜, 李鵬飛, 等. 結締組織病相關間質性肺疾病合并肺動脈高壓的預后模型開發與驗證. 中國呼吸與危重監護雜志, 2024, 23(9): 640-650.
21.	Perros F, Dorfmüller P, Montani D, et al. Pulmonary lymphoid neogenesis in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med, 2012, 185(3): 311-321.
22.	Bois MC, May AM, Vassallo R, et al. Morphometric study of pulmonary arterial changes in pulmonary langerhans cell histiocytosis. Arch Pathol Lab Med, 2018, 142(8): 929-937.
23.	Cottin V, Harari S, Humbert M, et al. Pulmonary hypertension in lymphangioleiomyomatosis: Characteristics in 20 patients. Eur Respir J, 2012, 40(3): 630-640.
24.	Dhont S, Zwaenepoel B, Vandecasteele E, et al. Pulmonary hypertension in interstitial lung disease: an area of unmet clinical need. ERJ Open Res, 2022, 8(4): 00272-02022.
25.	King CS, Brown AW, Shlobin OA, et al. Prevalence and impact of WHO group 3 pulmonary hypertension in advanced idiopathic nonspecific interstitial pneumonia. Eur Respir J, 2018, 52(1): 1800545.
26.	Oliveira RKF, Pereira CAC, Ramos RP, et al. A haemodynamic study of pulmonary hypertension in chronic hypersensitivity pneumonitis. Eur Respir J, 2014, 44(2): 415-424.
27.	Tyagi R, Kant S, Verma SK, et al. Spectrum of interstitial lung diseases and their association with pulmonary hypertension. Monaldi Arch Chest Dis, 2021, 92(3).
28.	Cottin V, Le Pavec J, Prévot G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J, 2010, 35(1): 105-111.
29.	Maher TM. Interstitial lung disease: a review. JAMA, 2024, 331(19): 1655-1665.
30.	Sonaglioni A, Cassandro R, Luisi F, et al. Correlation between doppler echocardiography and right heart catheterisation-derived systolic and mean pulmonary artery pressures: determinants of discrepancies between the two methods. Heart Lung Circ, 2021, 30(5): 656-664.
31.	Mandoli GE, Landra F, Chiantini B, et al. Tricuspid regurgitation velocity and mean pressure gradient for the prediction of pulmonary hypertension according to the new hemodynamic definition. Diagnostics (Basel), 2023, 13(16): 2619.
32.	Ben-Dor I, Kramer MR, Raccah A, et al. Echocardiography versus right-sided heart catheterization among lung transplantation candidates. Ann Thorac Surg, 2006, 81(3): 1056-1060.
33.	Keir GJ, Wort SJ, Kokosi M, et al. Pulmonary hypertension in interstitial lung disease: Limitations of echocardiography compared to cardiac catheterization. Respirology, 2018, 23(7): 687-694.
34.	Nowak J, Hudzik B, Jastrz?bski D, et al. Pulmonary hypertension in advanced lung diseases: Echocardiography as an important part of patient evaluation for lung transplantation. Clin Respir J, 2018, 12(3): 930-938.
35.	Andersen KH, Iversen M, Kjaergaard J, et al. Prevalence, predictors, and survival in pulmonary hypertension related to end-stage chronic obstructive pulmonary disease. J Heart Lung Transplant, 2012, 31(4): 373-380.
36.	Mukherjee M, Jani VP, Osgueritchian R, et al. Echocardiographic parameters and risk prediction in pulmonary arterial hypertension: Insights from the redefining pulmonary hypertension through pulmonary vascular disease phenomics network. Chest, 2025, 168(2): 502-519.
37.	Ruocco G, Cekorja B, Rottoli P, et al. Role of BNP and echo measurement for pulmonary hypertension recognition in patients with interstitial lung disease: An algorithm application model. Respir Med, 2015, 109(3): 406-415.
38.	Shin S, King CS, Puri N, et al. Pulmonary artery size as a predictor of outcomes in idiopathic pulmonary fibrosis. Eur Respir J, 2016, 47(5): 1445-1451.
39.	Fakharian A, Hamidi N, Hosseinloo BH, et al. Correlation between the pulmonary artery pressure measured in echocardiography and pulmonary artery diameter in the CT-scan of patients suffering from interstitial lung disease. Tanaffos, 2011, 10(3): 37-41.
40.	Nathan SD, du Bois RM, Albera C, et al. Validation of test performance characteristics and minimal clinically important difference of the 6-minute walk test in patients with idiopathic pulmonary fibrosis. Respir Med, 2015, 109(7): 914-922.
41.	Gonzalez-Hermosillo LM, Cueto-Robledo G, Roldan-Valadez E, et al. Right heart catheterization (RHC): A comprehensive review of provocation tests and hepatic hemodynamics in patients with pulmonary hypertension (PH). Curr Probl Cardiol, 2022, 47(12): 101351.
42.	Boerrigter BG, Waxman AB, Westerhof N, et al. Measuring central pulmonary pressures during exercise in COPD: How to cope with respiratory effects. Eur Respir J, 2014, 43(5): 1316-1325.
43.	Levy I, Horvath A, Azevedo M, et al. Phosphodiesterase function and endocrine cells: Links to human disease and roles in tumor development and treatment. Curr Opin Pharmacol, 2011, 11(6): 689-697.
44.	Ala M, Mohammad Jafari R, Dehpour AR. Sildenafil beyond erectile dysfunction and pulmonary arterial hypertension: Thinking about new indications. Fundam Clin Pharmacol, 2021, 35(2): 235-259.
45.	龔銘麗, 張居琪, 李文慧, 等. 西地那非治療新生兒持續性肺動脈高壓有效性和安全性分析. 中國臨床藥理學雜志, 2023, 39(20): 3005-3008.
46.	Idiopathic Pulmonary Fibrosis Clinical Research Network, Zisman DA, Schwarz M, et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med, 2010, 363(7): 620-628.
47.	Kolb M, Raghu G, Wells AU, et al. Nintedanib plus sildenafil in patients with idiopathic pulmonary fibrosis. N Engl J Med, 2018, 379(18): 1722-1731.
48.	Jerjes-Sánchez C, Glenn-Valdez H, Zayas N, et al. Riociguat in the treatment of pulmonary arterial hypertension. Arch Med Res, 2022, 53(4): 352-358.
49.	Benza RL, Langleben D, Hemnes AR, et al. Riociguat and the right ventricle in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eur Respir Rev, 2022, 31(166): 220061.
50.	Nathan SD, Behr J, Collard HR, et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): A randomised, placebo-controlled phase 2b study. Lancet Respir Med, 2019, 7(9): 780-790.
51.	Nathan SD, Cottin V, Behr J, et al. Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: a post hoc subgroup analysis of the RISE-IIP study. J Heart Lung Transplant, 2021, 40(6): 494-503.
52.	Nathan SD, Flaherty KR, Glassberg MK, et al. A randomized, double-blind, placebo-controlled study of pulsed, inhaled nitric oxide in subjects at risk of pulmonary hypertension associated with pulmonary fibrosis. Chest, 2020, 158(2): 637-645.
53.	Li HF, Wang JX, Xie ZF, et al. Bosentan and ambrisentan in the treatment of idiopathic pulmonary fibrosis: A meta-analysis. Eur Rev Med Pharmacol Sci, 2024, 28(3): 1183-1193.
54.	King TE, Behr J, Brown KK, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2008, 177(1): 75-81.
55.	Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med, 2013, 158(9): 641-649.
56.	Shlobin OA, Shen E, Wort SJ, et al. Pulmonary hypertension in the setting of interstitial lung disease: Approach to management and treatment. A consensus statement from the pulmonary vascular research institute's innovative drug development initiative-group 3 pulmonary hypertension. Pulm Circ, 2024, 14(1): e12310.
57.	Nathan SD, Johri S, Joly JM, et al. Survival analysis from the INCREASE study in PH-ILD: evaluating the impact of treatment crossover on overall mortality. Thorax, 2024, 79(4): 301-306.
58.	Weatherald J, Nathan SD, El-Kersh K, et al. Inhaled treprostinil in patients with pulmonary hypertension associated with interstitial lung disease with less severe haemodynamics: a post hoc analysis of the INCREASE study. BMJ Open Respir Res, 2024, 11(1): e002116.

1. Zhao N, Chen J, Zhang M, et al. PAH-specific therapy for pulmonary hypertension and interstitial lung disease: a systemic review and meta-analysis. Front Cardiovasc Med, 2022, 9: 992879.
2. Wells AU, Brown KK, Flaherty KR, et al. What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J, 2018, 51(5): 1800692.
3. Ruaro B, Baratella E, Confalonieri P, et al. High-resolution computed tomography: Lights and shadows in improving care for SSc-ILD patients. Diagnostics (Basel), 2021, 11(11): 1960.
4. Nathan SD, Barbera JA, Gaine SP, et al. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J, 2019, 53(1): 1801914.
5. King CS, Shlobin OA. The trouble with group 3 pulmonary hypertension in interstitial lung disease: Dilemmas in diagnosis and the conundrum of treatment. Chest, 2020, 158(4): 1651-1664.
6. Waxman AB, Elia D, Adir Y, et al. Recent advances in the management of pulmonary hypertension with interstitial lung disease. Eur Respir Rev, 2022, 31(165): 210220.
7. Sd C, J AG, As CS, et al. Pulmonary hypertension associated with idiopathic pulmonary fibrosis: Current and future perspectives. Can Respir J, 2017, 2017: 1430350.
8. Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease. N Engl J Med, 2021, 384(4): 325-334.
9. Nathan SD, Behr J, Cottin V, et al. Study design and rationale for the TETON phase 3, randomised, controlled clinical trials of inhaled treprostinil in the treatment of idiopathic pulmonary fibrosis. BMJ Open Respir Res, 2022, 9(1): e001310.
10. Johnson S, Sommer N, Cox-Flaherty K, et al. Pulmonary hypertension: a contemporary review. Am J Respir Crit Care Med, 2023, 208(5): 528-548.
11. Mocumbi A, Humbert M, Saxena A, et al. Pulmonary hypertension. Nat Rev Dis Primers, 2024, 10(1): 1.
12. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J, 2019, 53(1): 1801913.
13. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J, 2023, 61(1): 2200879.
14. Wijsenbeek M, Suzuki A, Maher TM. Interstitial lung diseases. Lancet, 2022, 400(10354): 769-786.
15. Braganza M, Shaw J, Solverson K, et al. A prospective evaluation of the diagnostic accuracy of the physical examination for pulmonary hypertension. Chest, 2019, 155(5): 982-990.
16. 徐婉婷, 江曉琴, 朱濤. 肺動脈高壓的治療進展. 中國呼吸與危重監護雜志, 2024, 23(4): 287-292.
17. Olsson KM, Corte TJ, Kamp JC, et al. Pulmonary hypertension associated with lung disease: new insights into pathomechanisms, diagnosis, and management. Lancet Respir Med, 2023, 11(9): 820-835.
18. Shlobin OA, Adir Y, Barbera JA, et al. Pulmonary hypertension associated with lung diseases. Eur Respir J, 2024, 64(4): 2401200.
19. Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev, 2018, 27(150): 180076.
20. 樊銘薇, 蔣天賜, 李鵬飛, 等. 結締組織病相關間質性肺疾病合并肺動脈高壓的預后模型開發與驗證. 中國呼吸與危重監護雜志, 2024, 23(9): 640-650.
21. Perros F, Dorfmüller P, Montani D, et al. Pulmonary lymphoid neogenesis in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med, 2012, 185(3): 311-321.
22. Bois MC, May AM, Vassallo R, et al. Morphometric study of pulmonary arterial changes in pulmonary langerhans cell histiocytosis. Arch Pathol Lab Med, 2018, 142(8): 929-937.
23. Cottin V, Harari S, Humbert M, et al. Pulmonary hypertension in lymphangioleiomyomatosis: Characteristics in 20 patients. Eur Respir J, 2012, 40(3): 630-640.
24. Dhont S, Zwaenepoel B, Vandecasteele E, et al. Pulmonary hypertension in interstitial lung disease: an area of unmet clinical need. ERJ Open Res, 2022, 8(4): 00272-02022.
25. King CS, Brown AW, Shlobin OA, et al. Prevalence and impact of WHO group 3 pulmonary hypertension in advanced idiopathic nonspecific interstitial pneumonia. Eur Respir J, 2018, 52(1): 1800545.
26. Oliveira RKF, Pereira CAC, Ramos RP, et al. A haemodynamic study of pulmonary hypertension in chronic hypersensitivity pneumonitis. Eur Respir J, 2014, 44(2): 415-424.
27. Tyagi R, Kant S, Verma SK, et al. Spectrum of interstitial lung diseases and their association with pulmonary hypertension. Monaldi Arch Chest Dis, 2021, 92(3).
28. Cottin V, Le Pavec J, Prévot G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J, 2010, 35(1): 105-111.
29. Maher TM. Interstitial lung disease: a review. JAMA, 2024, 331(19): 1655-1665.
30. Sonaglioni A, Cassandro R, Luisi F, et al. Correlation between doppler echocardiography and right heart catheterisation-derived systolic and mean pulmonary artery pressures: determinants of discrepancies between the two methods. Heart Lung Circ, 2021, 30(5): 656-664.
31. Mandoli GE, Landra F, Chiantini B, et al. Tricuspid regurgitation velocity and mean pressure gradient for the prediction of pulmonary hypertension according to the new hemodynamic definition. Diagnostics (Basel), 2023, 13(16): 2619.
32. Ben-Dor I, Kramer MR, Raccah A, et al. Echocardiography versus right-sided heart catheterization among lung transplantation candidates. Ann Thorac Surg, 2006, 81(3): 1056-1060.
33. Keir GJ, Wort SJ, Kokosi M, et al. Pulmonary hypertension in interstitial lung disease: Limitations of echocardiography compared to cardiac catheterization. Respirology, 2018, 23(7): 687-694.
34. Nowak J, Hudzik B, Jastrz?bski D, et al. Pulmonary hypertension in advanced lung diseases: Echocardiography as an important part of patient evaluation for lung transplantation. Clin Respir J, 2018, 12(3): 930-938.
35. Andersen KH, Iversen M, Kjaergaard J, et al. Prevalence, predictors, and survival in pulmonary hypertension related to end-stage chronic obstructive pulmonary disease. J Heart Lung Transplant, 2012, 31(4): 373-380.
36. Mukherjee M, Jani VP, Osgueritchian R, et al. Echocardiographic parameters and risk prediction in pulmonary arterial hypertension: Insights from the redefining pulmonary hypertension through pulmonary vascular disease phenomics network. Chest, 2025, 168(2): 502-519.
37. Ruocco G, Cekorja B, Rottoli P, et al. Role of BNP and echo measurement for pulmonary hypertension recognition in patients with interstitial lung disease: An algorithm application model. Respir Med, 2015, 109(3): 406-415.
38. Shin S, King CS, Puri N, et al. Pulmonary artery size as a predictor of outcomes in idiopathic pulmonary fibrosis. Eur Respir J, 2016, 47(5): 1445-1451.
39. Fakharian A, Hamidi N, Hosseinloo BH, et al. Correlation between the pulmonary artery pressure measured in echocardiography and pulmonary artery diameter in the CT-scan of patients suffering from interstitial lung disease. Tanaffos, 2011, 10(3): 37-41.
40. Nathan SD, du Bois RM, Albera C, et al. Validation of test performance characteristics and minimal clinically important difference of the 6-minute walk test in patients with idiopathic pulmonary fibrosis. Respir Med, 2015, 109(7): 914-922.
41. Gonzalez-Hermosillo LM, Cueto-Robledo G, Roldan-Valadez E, et al. Right heart catheterization (RHC): A comprehensive review of provocation tests and hepatic hemodynamics in patients with pulmonary hypertension (PH). Curr Probl Cardiol, 2022, 47(12): 101351.
42. Boerrigter BG, Waxman AB, Westerhof N, et al. Measuring central pulmonary pressures during exercise in COPD: How to cope with respiratory effects. Eur Respir J, 2014, 43(5): 1316-1325.
43. Levy I, Horvath A, Azevedo M, et al. Phosphodiesterase function and endocrine cells: Links to human disease and roles in tumor development and treatment. Curr Opin Pharmacol, 2011, 11(6): 689-697.
44. Ala M, Mohammad Jafari R, Dehpour AR. Sildenafil beyond erectile dysfunction and pulmonary arterial hypertension: Thinking about new indications. Fundam Clin Pharmacol, 2021, 35(2): 235-259.
45. 龔銘麗, 張居琪, 李文慧, 等. 西地那非治療新生兒持續性肺動脈高壓有效性和安全性分析. 中國臨床藥理學雜志, 2023, 39(20): 3005-3008.
46. Idiopathic Pulmonary Fibrosis Clinical Research Network, Zisman DA, Schwarz M, et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med, 2010, 363(7): 620-628.
47. Kolb M, Raghu G, Wells AU, et al. Nintedanib plus sildenafil in patients with idiopathic pulmonary fibrosis. N Engl J Med, 2018, 379(18): 1722-1731.
48. Jerjes-Sánchez C, Glenn-Valdez H, Zayas N, et al. Riociguat in the treatment of pulmonary arterial hypertension. Arch Med Res, 2022, 53(4): 352-358.
49. Benza RL, Langleben D, Hemnes AR, et al. Riociguat and the right ventricle in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eur Respir Rev, 2022, 31(166): 220061.
50. Nathan SD, Behr J, Collard HR, et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): A randomised, placebo-controlled phase 2b study. Lancet Respir Med, 2019, 7(9): 780-790.
51. Nathan SD, Cottin V, Behr J, et al. Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: a post hoc subgroup analysis of the RISE-IIP study. J Heart Lung Transplant, 2021, 40(6): 494-503.
52. Nathan SD, Flaherty KR, Glassberg MK, et al. A randomized, double-blind, placebo-controlled study of pulsed, inhaled nitric oxide in subjects at risk of pulmonary hypertension associated with pulmonary fibrosis. Chest, 2020, 158(2): 637-645.
53. Li HF, Wang JX, Xie ZF, et al. Bosentan and ambrisentan in the treatment of idiopathic pulmonary fibrosis: A meta-analysis. Eur Rev Med Pharmacol Sci, 2024, 28(3): 1183-1193.
54. King TE, Behr J, Brown KK, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2008, 177(1): 75-81.
55. Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med, 2013, 158(9): 641-649.
56. Shlobin OA, Shen E, Wort SJ, et al. Pulmonary hypertension in the setting of interstitial lung disease: Approach to management and treatment. A consensus statement from the pulmonary vascular research institute's innovative drug development initiative-group 3 pulmonary hypertension. Pulm Circ, 2024, 14(1): e12310.
57. Nathan SD, Johri S, Joly JM, et al. Survival analysis from the INCREASE study in PH-ILD: evaluating the impact of treatment crossover on overall mortality. Thorax, 2024, 79(4): 301-306.
58. Weatherald J, Nathan SD, El-Kersh K, et al. Inhaled treprostinil in patients with pulmonary hypertension associated with interstitial lung disease with less severe haemodynamics: a post hoc analysis of the INCREASE study. BMJ Open Respir Res, 2024, 11(1): e002116.

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間質性肺疾病合并肺動脈高壓的研究進展

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