Preview

PULMONOLOGIYA

Advanced search

Aspects of diagnosis and providing care for interstitial lung diseases in a specialized center

https://doi.org/10.18093/0869-0189-2024-34-6-920-929

Abstract

The problem of timely diagnosis of interstitial lung diseases (ILD) remains relevant today.

The aim. To describe the aspects of diagnosis, treatment, and providing care for ILD in a specialized center.

Results. During 2023, a panel of specialists examined 438 patients with ILD out of 2 373 (18.4%) at the prehospital stage who were referred for consultation to a specialized center. In the end, 181 patients with ILD were hospitalized for invasive diagnostic procedures and the decision upon prescribing antifibrotic therapy. The characteristics of patients with various clinical forms of ILD included in the register (n = 134) and the distribution of antifibrotic drugs are presented. The clinical and functional and computed tomography (CT) patterns in the patients receiving antifibrotic therapy (AFT) were analyzed.

Conclusion. The organizational, diagnostic and therapeutic algorithm and routing of patients with ILD on the basis of a specialized federal institution allows solving the problems of ILD diagnosis with high professionalism and successfully monitoring the patients on AFT.

About the Authors

I. V. Leshchenko
Federal State Budget Educational Institution of Higher Education “Ural State Medical University” of the Ministry of Health of the Russian Federation; Ural Federal Research Institute of Phthisiology and Pulmonology – a Branch of National Medical Research Center for Phthisiology, Pulmonology and Infectious Diseases, Healthcare Ministry of Russia
Russian Federation

Igor V. Leshсhenko - Doctor of Medicine, Professor; Professor, Department of Infectious Diseases, Phthisiology and Pulmonology, USMU; Chief Researcher, Ural Federal Research Institute of Phthisiology and Pulmonology – a BNMRC for Phthisiology, Pulmonology and Infectious Diseases, Healthcare Ministry of Russia; Scientific Director, Limited Liability Company “Novaya bol’nitsa” Clinical Association, Honored Doctor RF.

Ul. Repina 3, Ekaterinburg, 620028; ul. 22-go Parts’ezda 50, Ekaterinburg, 620039; ul. Zavodskaya 29, Ekaterinburg, 620109; tel.: (912) 288-28-23


Competing Interests:

The authors declare no conflict of interest



T. V. Glushkova
Ural Federal Research Institute of Phthisiology and Pulmonology – a Branch of National Medical Research Center for Phthisiology, Pulmonology and Infectious Diseases, Healthcare Ministry of Russia
Russian Federation

Tatiana V. Glushkova - Candidate of Medicine, Pulmonologist.

ul. 22-go Parts’ezda 50, Ekaterinburg, 620039; tel.: (343) 333-44-33


Competing Interests:

The authors declare no conflict of interest



N. M. Trifanova
Limited Liability Company “Novaya bol’nitsa” Clinical Association
Russian Federation

Natalya M. Trifanova - Candidate of Medicine, Pulmonologist.

Zavodskaya 29, Ekaterinburg, 620109; tel.: (908) 900-55-92


Competing Interests:

The authors declare no conflict of interest



N. A. Esaulova
Ural Federal Research Institute of Phthisiology and Pulmonology – a Branch of National Medical Research Center for Phthisiology, Pulmonology and Infectious Diseases, Healthcare Ministry of Russia
Russian Federation

Natalia A. Esaulova - Candidate of Medicine, Head of the Department of Differential Diagnosis of Tuberculosis.

ul. 22-go Parts’ezda 50, Ekaterinburg, 620039; tel.: (922) 204-04-97


Competing Interests:

The authors declare no conflict of interest



References

1. Travis W.D., Costabel U., Hansell D.M. et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am. J. Respir. Crit. Care Med. 2013; 188 (6): 733–748. DOI: 10.1164/rccm.201308-1483ST.

2. Raghu G., Collard H.R., Egan J.J. 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 (6): 788–824. DOI: 10.1164/rccm.2009-040GL.

3. Flaherty K.R., Brown K.K., Wells A.U. et al. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir. Res. 2017; 4 (1): e000212. DOI: 10.1136/bmjresp-2017-000212.

4. Wells A.U., Brown K.K., Flaherty K.R. 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. DOI: 10.1183/13993003.00692-2018.

5. Raghu G., Remy-Jardin M., Richeldi L. et al. 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): e18–47. DOI: 10.1164/rccm.202202-0399ST.

6. Cottin V., Hirani N.A., Hotchkin D.L. et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur. Respir. Rev. 2018; 27 (150): 180076. DOI: 10.1183/16000617.0076-2018.

7. Rajan S.K., Cottin V., Dhar R. et al. Progressive pulmonary fibrosis: an Expert Group Consensus Statement. Eur. Respir. J. 2023; 30; 61 (3): 2103187. DOI: 10.1183/13993003.03187-2021.

8. Meyer K.C., Raghu G., Baughman R.P. et al. An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am. J. Respir. Crit. Care Med. 2012; 185 (9): 1004–1014. DOI: 10.1164/rccm.201202-0320st.

9. Raghu G., Remy-Jardin M., Ryerson Ch. J. et al. Diagnosis of hypersensitivity pneumonitis in adults. An official ATS/JRS/ALAT clinical practice guideline. Am. J. Respir. Crit. Care Med. 2020; 202 (3): e36–69. DOI: 10.1164/rccm.202005-2032st.

10. Wijsenbeek M., Kreuter M., Olson A. et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr. Med. Res. Opin. 2019; 35 (11): 2015–2024. DOI: 10.1080/03007995.2019.1647040.

11. Faverio P., Piluso M., De Giacomi F. et al. Progressive fibrosing interstitial lung diseases: prevalence and characterization in two Italian Referral Centers. Respiration. 2020; 99 (10): 838–845. DOI: 10.1159/000509556.

12. Nasser M., Larrieu S., Si-Mohamed S. et al. Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS study). Eur. Respir. J. 2021: 11; 57 (2): 2002718. DOI: 10.1183/13993003.02718-2020.

13. Ignatova G.L., Antonov V.N., Blinova E.V., Belsner M.S. [The effectiveness of early administration of antifibrotic therapy as a reflection of properly organized medical care for patients with progressive pulmonary fibrosis]. Pul’monologiya. 2024; 34 (1): 115–120. DOI: 10.18093/0869-0189-2024-34-1-115-120 (in Russian).

14. Noble P.W., Albera C., Bradford W.Z. et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet Respir. Med. 2011; 377 (9779): 1760–1769. DOI: 10.1016/S0140-6736(11)60405-4.

15. Taniguchi H., Ebina M., Kondoh Y. et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur. Respir. J. 2010; 35 (4): 821–829. DOI: 10.1183/09031936.00005209.

16. King T.E. Jr., Bradford W.Z., Castro-Bernardini S. et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N. Engl. J. Med. 2014; 370 (22): 2083–2092. DOI: 10.1056/NEJMoa1402582.

17. Stepanyan I.E., Zaytseva A.S., Shergina E.A. [Long-term pirfenidone treatment in the Russian patients with idiopathic pulmonary fibrosis]. Klinicheskaya farmakologiya i terapiya. 2018, 27 (4): 77–80. Available at: https://clinpharm-journal.ru/articles/2018-4/opyt-dlitelnogo-primeneniya-pirfenidona-u-patsientov-s-idiopaticheskim-legochnym-fibrozom/ (in Russian).

18. Glassberg M.K., Wijsenbeek M.S., Gilberg F. et al. Effect of pirfenidone on breathlessness in patients with idiopathic pulmonary fibrosis. Eur. Respir. J. 2019; 54 (3): 1900399. DOI: 10.1183/13993003.00399-2019.

19. Marcos Ribes B., Sancho-Chust J.N., Talens A. et al. Effectiveness and safety of pirfenidone for idiopathic pulmonary fibrosis. Eur. J. Hosp. Pharm. 2020; 27 (6): 350–354. DOI: 10.1136/ejhpharm-2018-001806.

20. Solomon J.J., Danoff S.K., Woodhead F.A. et al. Safety, tolerability, and efficacy of pirfenidone in patients with rheumatoid arthritis-associated interstitial lung disease: a randomised, double-blind, placebo-controlled, phase 2 study. Lancet Respir. Med. 2023; 11 (1): 87–96. DOI: 10.1016/S2213-2600(22)00260-0.

21. Lancaster L.H., de Andrade J.A., Zibrak J.D. et al. Pirfenidone safety and adverse event management in idiopathic pulmonary fibrosis. Eur. Respir. Rev. 2017; 26 (146): 170057. DOI: 10.1183/16000617.0057-2017.


Supplementary files

Review

For citations:


Leshchenko I.V., Glushkova T.V., Trifanova N.M., Esaulova N.A. Aspects of diagnosis and providing care for interstitial lung diseases in a specialized center. PULMONOLOGIYA. 2024;34(6):920-929. (In Russ.) https://doi.org/10.18093/0869-0189-2024-34-6-920-929

Views: 188


ISSN 0869-0189 (Print)
ISSN 2541-9617 (Online)