Chronic fibrosing progressing interstitial lung disease: a decision of Multidisciplinary Expert Board
https://doi.org/10.18093/0869-0189-2021-31-4-505-510
Abstract
Introduction. The natural course of some interstitial lung diseases (ILD) is characterized by progressive fibrosing phenotype resembling idiopathic pulmonary fibrosis (IPF). Until recently, the antifibrotic drug nintedanib was approved for treatment of the only fibrosing ILD which was IPF. A new indication for this drug which has been registered in Russian Federation in 2021 includes other fibrosing ILDs with progressive phenotype (PF-ILDs) and ILD associated with systemic scleroderma (SS-ILD).
The aim of this publication is to describe general considerations of the decision of Multidisciplinary Expert Board on diagnosis and treatment of PF-ILDs including SS-ILD.
Results. According to the extension in nintedanib use mentioned above, the Expert Board created an algorithm for diagnosis and treatment of patients with PF-ILDs and criteria for nuntedanib administration in PF-ILDs.
Conclusion. Antifibrotic therapy is needed for patients with PF-ILDs with the failure of the stanrard therapy. In those patients antifibrotic treatment should be initiated as early as possible to better preserve the lung function.
Keywords
About the Authors
S. N. AvdeevRussian Federation
Sergey N. Avdeev, Doctor of Medicine, Professor, Corresponding Member of Russian Academy of Sciences, Head of Department of Pulmonology, N.V.Sklifosovskiy Institute of Clinical Medicine
ul. Trubetskaya 8, build. 2, Moscow, 119991
SPIN-code: 1645-5524
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
S. Yu. Chikina
Russian Federation
Svetlana Yu. Chikina, Candidate of Medicine, Assistant Lecturer, Department of Pulmonology, N.V.Sklifosovskiy Institute of Clinical Medicine
ul. Trubetskaya 8, build. 2, Moscow, 119991
SPIN-code: 4463-8203
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
I. E. Tyurin
Russian Federation
Igor’ E. Tyurin, Doctor of Medicine, Professor, Head of Department of Radiology
ul. Barrikadnaya 2/1, Moscow, 123995
SPIN-code: 6499-2398
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
A. S. Belevskiy
Russian Federation
Andrey S. Belevskiy, Doctor of Medicine, Professor, Head of Department of Pulmonology, Faculty of Postgraduate Physician Training, N.I.Pirogov Federal Russian National Research Medical University, Healthcare Ministry of Russia; Chairman of Russian Respiratory Society; Chief Pulmonologist, Moscow Healthcare Department
ul. Ostrovityanova 1, Moscow, 117997
SPIN-code: 7313-8885
Competing Interests:
lecturing for Boehringer Ingelheim
S. A. Terpigorev
Russian Federation
Stanislav A. Terpigorev, Doctor of Medicine, Head of Department of Occupational Diseases and Medical Expertise
ul. Shchepkina 61/2, Moscow, 129110
SPIN-code: 9404-3122
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
L. P. Anan’yeva
Russian Federation
Lidiya P. Anan’eva, Doctor of Medicine, Professor, Head of Laboratory of Microcirculation and Inflammation
Kashirskoe shosse 34A, Moscow, 115522
Competing Interests:
no
A. A. Vizel’
Russian Federation
Aleksandr A. Vizel’, Doctor of Medicine, Professor, Head of Department of Phthisiology and Pulmonology
SPINcode: 5918-5465;
Author ID: 195447
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
M. V. Boldina
Russian Federation
Marina V. Boldina, Candidate of Medicine, Associate Professor, Department of Therapy and Cardiology
ph. Minina i Pozharskogo 10/1, Nizhniy Novgorod, 603950
SPIN-code: 8345-0153;
Author ID: 561563
Competing Interests:
Рarticipation in clinical research and lecturing for Boehringer Ingelheim
I. V. Demko
Russian Federation
Irina V. Demko, Doctor of Medicine, Professor, Head of Department No.2 of Internal Medicine and Postgraduate Physician Training Course
ul. Partizana Zheleznyaka 1, Krasnoyarsk, 660022
SPIN-code: 6520- 3233;
Author ID: 608300
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
I. V. Leshchenko
Russian Federation
Igor’ V. Leshchenko, Doctor of Medicine, Professor, Department of Phthisiology and Pulmonology, Ural Federal State Medical University, Healthcare Ministry of Russia; Chief Researcher, 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; Academic Advisor of “Novaya bol’nitsa” Clinical Associationon
ul. Repina 3, Ekaterinburg, 620028,
ul. 22nd Parts’ezda 50, Sverdlovskaya obl., Ekaterinburg, 620039,
ul. Zavodskaya 29, Ekaterinburg, 620109
SPIN-code: 1851- 8986;
Author ID: 367678
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
I. N. Trofimenko
Russian Federation
Irina N. Trofimenko, Doctor of Medicine, Associate Professor, Head of Department of Clinical Allergology and Pulmonology
mkr. Yubileynyy 100, Irkutsk, 664079
SPIN-code: 9345-4572;
Author ID: 636656
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
M. F. Kinyaykin
Russian Federation
Mihail F. Kiniaikin, Candidate of Medicine, Associate Professor, Institute of Therapy and Instrumental Diagnostics, Federal State Budget Educational Institution of Higher Education «Pacific State Medical University», Healthcare Ministry of Russia, Head of the Regional Pulmonary Center, Primorsky Regional Clinical Hospital No.1
рrosp. Ostryakova 2, Vladivostok, 690002,
ul. Aleutskaya 57, Vladivostok, 690091
SPIN-code: 2069-6636;
Author ID: 571691
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
I. E. Stepanyan
Russian Federation
Igor’ E. Stepanyan, Doctor of Medicine, Professor, Leading Researcher, Division of Differential Diagnosis of Pulmonary Tuberculosis and Extracorporeal Therapeutic Methods
Yauzskaya alleya 2, Moscow, 107564
SPIN-code: 8854-5951;
Author ID: 443264
Competing Interests:
Writing articles, participating in clinical trials and lecturing for Boehringer Ingelheim
A. S. Zaytseva
Russian Federation
Anna S. Zaytseva, Candidate of medicine, Head of Therapeutic Department No.4, Senoir Researcher, Division of Differential Diagnosis of Tuberculosis and Extracorporeal Therapeutic Methods
Yauzskaya alleya 2, Moscow, 107564
SPIN-code: 8986-4397;
Author ID: 707405
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
D. V. Petrov
Russian Federation
Dmitriy V. Petrov, pulmonologist
ul. Pereleta 7, Omsk, 644112
SPIN-code: 1721-4950;
Author ID: 732897
Competing Interests:
Writing articles and giving lectures for Boehringer Ingelheim
References
1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am. J. Respir. Crit. Care Med. 2002; 165 (2): 277–304. DOI: 10.1164/ajrccm.165.
2. ats01. 2. George P.M., Spagnolo P., Kreuter M. et al. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities. Lancet Respir. Med. 2020; 8 (9): 925–934. DOI: 10.1016/S2213-2600(20)30355-6.
3. Wijsenbeek M., Cottin V. Spectrum of fibrotic lung diseases. N. Engl. J. Med. 2020; 383 (10): 958–968. DOI: 10.1056/NEJMra2005230.
4. De Sadeleer L.J., Hermans F., De Dycker E. et al. Effects of corticosteroid treatment and antigen avoidance in a large hypersensitivity pneumonitis cohort: A single-centre cohort study. J. Clin. Med. 2019; 8 (1): 14. DOI: 10.3390/jcm8010014.
5. Fischer A., Distler J. Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases. Clin. Rheumatol. 2019; 38 (10): 2673–2681. DOI: 10.1007/s10067-019-04720-0.
6. Brown K.K., Martinez F.J., Walsh S.L.F. et al. The natural history of progressive fibrosing interstitial lung diseases. Eur. Respir. J. 2020; 55 (6): 2000085. DOI: 10.1183/13993003.00085-2020.
7. Kolb M., Richeldi L., Behr J. et al. Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume. Thorax. 2017; 72 (4): 340–346. DOI: 10.1136/thoraxjnl-2016-208710.
8. Distler O., Highland K.B., Gahlemann M. et al. Nintedanib for systemic sclerosis – associated interstitial lung disease. N. Engl. J. Med. 2019; 380 (26): 2518–2528. DOI: 10.1056/NEJMoa1903076.
9. Flaherty K.R., Wells A.U., Cottin V. et al. Nintedanib in progressive fibrosing interstitial lung diseases. N. Engl. J. Med. 2019; 381 (18): 1718–1727. DOI: 10.1056/NEJMoa1908681.
10. Russian Respiratory Society. [Idiopathic Pulmonary Fibrosis: Federal Clinical Guidelines]. 2016. Available at: https://www.volgmed.ru/uploads/files/2018-10/90708-idiopaticheskij_legochnyj_fibroz_2016.pdf [Accessed: June 21, 2021] (in Russian).
Review
For citations:
Avdeev S.N., Chikina S.Yu., Tyurin I.E., Belevskiy A.S., Terpigorev S.A., Anan’yeva L.P., Vizel’ A.A., Boldina M.V., Demko I.V., Leshchenko I.V., Trofimenko I.N., Kinyaykin M.F., Stepanyan I.E., Zaytseva A.S., Petrov D.V. Chronic fibrosing progressing interstitial lung disease: a decision of Multidisciplinary Expert Board. PULMONOLOGIYA. 2021;31(4):505-510. (In Russ.) https://doi.org/10.18093/0869-0189-2021-31-4-505-510