Preview

Bulletin Physiology and Pathology of Respiration

Advanced search

Clinical significance of assessing unventilated volume and ventilation heterogeneity in patients with combined pulmonary fibrosis and emphysema

https://doi.org/10.36604/1998-5029-2026-100-9-21

Abstract

Aim. To quantify the parameters of unventilated lung volume and pulmonary ventilation heterogeneity and study their clinical significance in patients with a combination of idiopathic pulmonary fibrosis and pulmonary emphysema (CPFE).

Materials and methods. The case histories of 56 patients with CPFE and 48 patients with idiopathic pulmonary fibrosis (IPF) were analyzed. The study of respiratory function included the measurement of static lung volumes and capacities, speed indicators and diffusion capacity of the lungs. The unventilated volume (ΔTLC) and the fraction of non-ventilated space (PCF) were also analyzed.

Results. The respiratory mechanics parameters in the group of patients with CPFE were significantly higher compared to patients with IPF. The ratio of forced expiration volume in the first second (FEV1) to vital capacity (VC), (median – 0.76) and FEV1 to forced VC (FVC), (median – 0.78) parameters were lower, but in most cases (89.3%) remained normal. The values of ΔTLC and PCF were significantly higher in the group of patients with CPFE (p < 0.001), while statistically significant relationships were found between these parameters and the parameters characterizing bronchial patency (FEV1/VC (p = 0.001), FEV1/FVC (p = 0.003)). With an increase in ΔTLC of more than 500 ml, pulmonary hypertension was significantly more common (OR – 3.102, p = 0.045), and an increase in PCF of more than 15% was statistically more often detected before the onset of death (OR – 7.800, p < 0.001).

Conclusion. Our data indicate the importance of assessing ΔTLC and PCF in the complex diagnostics of the external respiratory system in the CPFE. Their relationships with other indices of airway patency, as well as the incidence of pulmonary hypertension and fatal outcomes, may be an important link in determining the volume of therapy prescribed to these patients.

About the Authors

N. V. Markov
Federal State Budgetary Educational Institution of Higher Education Academician I.P. Pavlov First St. Petersburg State Medical University of the Ministry of Healthcare of Russian Federation
Russian Federation

Nikita V. Markov, MD, Pulmonologist, Department of Respiratory Therapy, Research Institute of Interstitial and Orphan Lung Diseases

6-8 L'va Tolstogo Str., St. Petersburg, 197022



N. A. Khodorik
Federal State Budgetary Educational Institution of Higher Education Academician I.P. Pavlov First St. Petersburg State Medical University of the Ministry of Healthcare of Russian Federation
Russian Federation

Natalia A. Khodorik, MD, PhD (Med.), DSc (Med.), Associate Professor, Department of Pulmonology, Faculty of Postgraduate Education

6-8 L'va Tolstogo Str., St. Petersburg, 197022



I. D. Rabik
Federal State Budgetary Educational Institution of Higher Education Academician I.P. Pavlov First St. Petersburg State Medical University of the Ministry of Healthcare of Russian Federation
Russian Federation

Iulia D. Rabik, MD, PhD (Med.), Associate Professor, Department of Functional Diagnostics; Head of Functional Diagnostics Unit No. 2

6-8 L'va Tolstogo Str., St. Petersburg, 197022



M. M. Ilkovich
Federal State Budgetary Educational Institution of Higher Education Academician I.P. Pavlov First St. Petersburg State Medical University of the Ministry of Healthcare of Russian Federation
Russian Federation

Mikhail M. Ilkovich, MD, PhD (Med.), DSc (Med.), Professor, Head of the Department of Pulmonology, Faculty of Postgraduate Education; Director of the Research Institute of Interstitial and Orphan Lung Diseases

6-8 L'va Tolstogo Str., St. Petersburg, 197022



References

1. Cottin V., Selman M., Inoue Y., Wong A.W., Corte T.J., Flaherty K.R., Han M.K., Jacob J., Johannson K.A., Kitaichi M., Lee J.S., Agusti A., Antoniou K.M., Bianchi P., Caro F., Florenzano M., Galvin L., Iwasawa T., Martinez F.J., Morgan R.L., Myers J.L., Nicholson A.G., Occhipinti M., Poletti V., Salisbury M.L., Sin D.D., Sverzellati N., Tonia T., Valenzuela C., Ryerson C.J., Wells A.U. Syndrome of combined pulmonary fibrosis and emphysema: an official ATS/ERS/JRS/ALAT Research Statement. Am. J. Respir. Crit. Care Med. 2022; 206(4):7–41. https://doi.org/10.1164/rccm.202206-1041ST

2. Cottin V., Cordier J.F. The syndrome of combined pulmonary fibrosis and emphysema. Chest 2009; 136(1):1–2. https://doi.org/10.1378/chest.09-0538

3. Cottin V., Nunes H., Brillet P.Y., Delaval P., Devouassoux G., Tillie-Leblond I., Israel-Biet D., Court-Fortune I., Valeyre D., Cordier J.F. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur. Respir. J. 2005; 26(4):586–593. https://doi.org/10.1183/09031936.05.00021005

4. Avdeev S.N., Aisanov Z.R., Belevskiy A.S., Ilkovich M.M., Kogan E.A., Merzhoeva Z.M., Petrov D.V., Samsonova M.V., Terpigorev S.A., Trushenko N.V., Trofimenko I.N., Tyurin I.E., Chernyaev A.L., Chernyak B.A., Chernyak A.V., ChikinaS.Yu., Chuchalin A.G., Shmelev E.I. [Federal clinical guidelines on diagnosis and treatment of idiopathic pulmonary fibrosis]. Pul’monologiya = Pulmonology 2022; 32(3):473–495 (in Russian). https://doi.org/10.18093/0869-0189-2022-32-3-473-495

5. Ilkovich M.M., editor. [Diffuse parenchymal lung diseases]. Moscow: GEOTAR-Media; 2021 (in Russian). ISBN: 978-5-9704-5908-9.

6. Ilkovich M.M., Novikova L.N., Speranskaya A.A., Dvorakovskaya I.V. [Progressive fibrosing lung disease. Discussion aspects of the problem: A review]. Terapevticheskiy arkhiv = Therapeutic Archive 2023; 95(3):255–259 (in Russian). https://doi.org/10.26442/00403660.2023.03.202075

7. Ilkovich M.M., Novikova L.N. [Idiopathic interstitial pneumonias]. Terapevticheskiy arkhiv = Therapeutic Archive 2021; 93(3):333–336 (in Russian). https://doi.org/10.26442/00403660.2021.03.200660

8. Jankowich M. D., Rounds S. Combined pulmonary fibrosis and emphysema alters physiology but has similar mortality to pulmonary fibrosis without emphysema. Lung 2010; 188(5):365–373. https://doi.org/10.1007/s00408-010-9251-6

9. Amariei D.E., Dodia N., Deepak J., Hines S.E., Galvin J.R., Atamas S.P., Todd N.W. Combined pulmonary fibrosis and emphysema: pulmonary function testing and a pathophysiology perspective. Medicina (Kaunas) 2019; 55(9):580. https://doi.org/10.3390/medicina55090580

10. Cano-Jiménez E., Hernández González F., Peloche G.B. Comorbidities and complications in idiopathic pulmonary fibrosis. Med. Sci. (Basel) 2018; 6(3):71. https://doi.org/10.3390/medsci6030071

11. Akagi T., Matsumoto T., Harada T., Tanaka M., Kuraki T., Fujita M., Watanabe K. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respir. Med. 2009; 103(8):1209–1215. https://doi.org/10.1016/j.rmed.2009.02.001

12. Kitaguchi Y., Fujimoto K., Hayashi R., Hanaoka M., Honda T., Kubo K. Annual changes in pulmonary function in combined pulmonary fibrosis and emphysema: over a 5-year follow-up. Respir. Med. 2013; 107(12):1986–1992. https://doi.org/10.1016/j.rmed.2013.06.015

13. Çiftci F., Gülpınar B., Atasoy Ç., Kayacan O., Saryal S. Combined pulmonary fibrosis and emphysema: How does cohabitation affect respiratory functions? Adv. Med. Sci. 2019; 64(2):285–291. https://doi.org/10.1016/j.advms.2019.03.005

14. Brodskaya O.N. [The combination of pulmonary fibrosis and emphysema – comorbidity or independent disease]. Prakticheskaya pul'monologiya = Practical Pulmonology 2018; 4:61–66 (in Russian).

15. Grippi M.A., editor. [Pathophysiology of the lungs]. Moscow: Izdatel'stvo BINOM; 2005 (in Russian). ISBN: 5- 7989-0131-9.

16. Shik L.L., Kanaev N.N., editors. [Guidelines for clinical respiratory physiology]. Leningrad: Meditsina; 1980 (in Russian).

17. Schaanning C.G., Gulsvik A. Accuracy and precision of helium dilution technique and body plethysmography in measuring lung volumes. Scand. J. Clin. Lab. Invest. 1973; 32(3):271–277. https://doi.org/10.3109/00365517309082471

18. Wade J.F.3rd., Mortenson R., Irvin C.G. Physiologic evaluation of bullous emphysema. Chest 1991; 100(4):1151– 1154. https://doi.org/10.1378/chest.100.4.1151

19. O'Donnell C.R., Bankier A.A., Stiebellehner L., Reilly J.J., Brown R., Loring S.H. Comparison of plethysmographic and helium dilution lung volumes: which is best for COPD? Chest 2010; 137(5):1108–1115. https://doi.org/10.1378/chest.09-1504

20. Cazzola M., Rogliani P., Curradi G., Segreti A., Ciaprini C., Pezzuto G., Saltini C. A pilot comparison of helium dilution and plethysmographic lung volumes to assess the impact of a long-acting bronchodilator on lung hyperinflation in COPD. Pulm. Pharmacol. Ther. 2009; 22(6):522–525. https://doi.org/10.1016/j.pupt.2009.05.005

21. D'Ascanio M., Viccaro F., Calabrò N., Guerrieri G., Salvucci C., Pizzirusso D., Mancini R., De Vitis C., Pezzuto A., Ricci A. Assessing static lung hyperinflation by whole-body plethysmography, helium dilution, and Impulse Oscillometry System (IOS) in patients with COPD. Int. J. Chron. Obstruct. Pulmon. Dis. 2020; 15:2583–2589. https://doi.org/10.2147/COPD.S264261

22. Kameneva M.Yu. [Syndromes of gas exchange abnormalities in patients with interstitial lung diseases]. Bylleten' fiziologii i patologii dykhaniya = Bulletin Physiology and Pathology of Respiration 2015; 56:14–20 (in Russian).

23. Neder J.A., O'Donnell C.D., Cory J., Langer D., Ciavaglia C.E., Ling Y., Webb K.A., O'Donnell D.E. Ventilation distribution heterogeneity at rest as a marker of exercise impairment in mild-to-advanced COPD. COPD 2015; 12(3):252– 259. https://doi.org/10.3109/15412555.2014.948997

24. Roberts C. M., MacRae K. D., Seed W. A. Multi-breath and single breath helium dilution lung volumes as a test of airway obstruction. Eur. Respir. J. 1990; 3(5):515–520.

25. Punjabi N.M., Shade D., Wise R.A. Correction of single-breath helium lung volumes in patients with airflow obstruction. Chest 1998; 114(3):907–918. https://doi.org/10.1378/chest.114.3.907

26. Agustí A., Celli B.R., Criner G.J., Halpin D., Anzueto A., Barnes P., Bourbeau J., Han M.K., Martinez F.J., Montes de Oca M., Mortimer K., Papi A., Pavord I., Roche N., Salvi S., Sin D.D., Singh D., Stockley R., López Varela M.V., Wedzicha J.A., Vogelmeier C.F. Global initiative for chronic obstructive lung disease 2023 report: GOLD executive summary. Am. J. Respir. Crit. Care Med. 2023; 207(7):819–837. https://doi.org/10.1164/rccm.202301-0106PP

27. Raghu G., Remy-Jardin M., Richeldi L., Thomson C.C., Inoue Y., Johkoh T., Kreuter M., Lynch D.A., Maher T.M., Martinez F.J., Molina-Molina M., Myers J.L., Nicholson A.G., Ryerson C..J, Strek M.E., Troy L.K., Wijsenbeek M., Mammen M.J., Hossain T., Bissell B.D., Herman D.D., Hon S.M., Kheir F., Khor Y.H., Macrea M., Antoniou K.M., Bouros D., Buendia-Roldan I., Caro F., Crestani B., Ho L., Morisset J., Olson A.L., Podolanczuk A., Poletti V., Selman M., Ewing T., Jones S., Knight S.L., Ghazipura M., Wilson K.C. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am. J. Respir. Crit. Care Med. 2022; 205(9):18–47. https://doi.org/10.1164/rccm.202202-0399ST

28. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am. J. Respir. Crit. Care Med. 2000; 161(2 Pt1):646–664. https://doi.org/10.1164/ajrccm.161.2.ats3-00

29. Avdeev S.N. [Current approach to diagnosis and therapy of pulmonary hypertension in patients with chronic obstructive pulmonary disease]. Pul’monologiya = Pulmonology 2009; 1:90–101 (in Russian).


Review

For citations:


Markov N.V., Khodorik N.A., Rabik I.D., Ilkovich M.M. Clinical significance of assessing unventilated volume and ventilation heterogeneity in patients with combined pulmonary fibrosis and emphysema. Bulletin Physiology and Pathology of Respiration. 2026;(100):9-21. (In Russ.) https://doi.org/10.36604/1998-5029-2026-100-9-21

Views: 41

JATS XML


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 1998-5029 (Print)