Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis

Wojciech J Piotrowski¹, Magdalena M Martusewicz-Boros², Adam J Białas³, Adam Barczyk⁴, Bogdan Batko⁵, Katarzyna Błasińska⁶, Piotr W Boros⁷, Katarzyna Górska⁸, Piotr Grzanka⁹, Ewa Jassem¹⁰, Dariusz Jastrzębski¹¹, Janina Kaczyńska¹², Otylia Kowal-Bielecka¹³, Eugeniusz Kucharz¹⁴, Jan Kuś¹⁵, Barbara Kuźnar-Kamińska¹⁶, Brygida Kwiatkowska¹⁷, Renata Langfort¹⁸, Katarzyna Lewandowska¹⁵, Barbara Mackiewicz¹⁹, Sebastian Majewski¹, Joanna Makowska²⁰, Joanna Miłkowska-Dymanowska¹, Elżbieta Puścińska²¹, Alicja Siemińska²², Małgorzata Sobiecka¹⁵, Renata Anna Soroka-Dąda²³, Małgorzata Szołkowska¹⁸, Elżbieta Wiatr², Dariusz Ziora¹¹, Paweł Śliwiński²¹

Affiliations

¹ Department of Pneumology, Medical University of Lodz, 90-153 Lodz, Poland.
² 3rd Lung Diseases and Oncology Department, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
³ Department of Pathobiology of Respiratory Diseases, Medical University of Lodz, 90-153 Lodz, Poland.
⁴ Department of Pneumonology, School of Medicine in Katowice, Medical University of Silesia, 40-635 Katowice, Poland.
⁵ Department of Rheumatology and Immunology, Faculty of Medicine and Health Sciences, Andrzej Frycz Modrzewski University, 30-705 Krakow, Poland.
⁶ Department of Radiology, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
⁷ Lung Pathophysiology Department, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
⁸ Department of Internal Medicine, Pulmonary Diseases and Allergy, Medical University of Warsaw, 02-091 Warsaw, Poland.
⁹ Department of Radiology, Voivodeship Hospital in Opole, 45-061 Opole, Poland.
¹⁰ Department of Allergology and Pneumonology, Medical University of Gdansk, 80-214 Gdańsk, Poland.
¹¹ Department of Lung Diseases and Tuberculosis, Medical University of Silesia, 41-803 Zabrze, Poland.
¹² Patient, not affiliated, 07-420 Kadzidlo, Poland.
¹³ Department of Rheumatology and Internal Medicine, Medical University of Białystok, 15-276 Białystok, Poland.
¹⁴ Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia, 40-635 Katowice, Poland.
¹⁵ 1st Lung Diseases Department, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
¹⁶ Department of Pulmonology, Allergology and Respiratory Oncology, University of Medical Sciences in Poznan, 61-701 Poznan, Poland.
¹⁷ Department of Rheumatology, Eleonora Reicher Rheumatology Institute, 02-637 Warszawa, Poland.
¹⁸ Department of Pathology, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warszawa, Poland.
¹⁹ Department of Pneumonology, Oncology and Allergology, Medical University, Lublin, 20-090 Lublin, Poland.
²⁰ Department of Rheumatology, Medical University of Lodz, 92-213 Lodz, Poland.
²¹ 2nd Department of Respiratory Medicine, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
²² Department of Allergology, Medical University of Gdańsk, 80-214 Gdansk, Poland.
²³ Patient, not affiliated, 02-673 Warsaw, Poland.

PMID: 36285980
PMCID: PMC9717335
DOI: 10.3390/arm90050052

Review

Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis

Wojciech J Piotrowski et al. Adv Respir Med. 2022.

. 2022 Oct 4;90(5):425-450.

doi: 10.3390/arm90050052.

Authors

Affiliations

¹ Department of Pneumology, Medical University of Lodz, 90-153 Lodz, Poland.
² 3rd Lung Diseases and Oncology Department, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
³ Department of Pathobiology of Respiratory Diseases, Medical University of Lodz, 90-153 Lodz, Poland.
⁴ Department of Pneumonology, School of Medicine in Katowice, Medical University of Silesia, 40-635 Katowice, Poland.
⁵ Department of Rheumatology and Immunology, Faculty of Medicine and Health Sciences, Andrzej Frycz Modrzewski University, 30-705 Krakow, Poland.
⁶ Department of Radiology, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
⁷ Lung Pathophysiology Department, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
⁸ Department of Internal Medicine, Pulmonary Diseases and Allergy, Medical University of Warsaw, 02-091 Warsaw, Poland.
⁹ Department of Radiology, Voivodeship Hospital in Opole, 45-061 Opole, Poland.
¹⁰ Department of Allergology and Pneumonology, Medical University of Gdansk, 80-214 Gdańsk, Poland.
¹¹ Department of Lung Diseases and Tuberculosis, Medical University of Silesia, 41-803 Zabrze, Poland.
¹² Patient, not affiliated, 07-420 Kadzidlo, Poland.
¹³ Department of Rheumatology and Internal Medicine, Medical University of Białystok, 15-276 Białystok, Poland.
¹⁴ Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia, 40-635 Katowice, Poland.
¹⁵ 1st Lung Diseases Department, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
¹⁶ Department of Pulmonology, Allergology and Respiratory Oncology, University of Medical Sciences in Poznan, 61-701 Poznan, Poland.
¹⁷ Department of Rheumatology, Eleonora Reicher Rheumatology Institute, 02-637 Warszawa, Poland.
¹⁸ Department of Pathology, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warszawa, Poland.
¹⁹ Department of Pneumonology, Oncology and Allergology, Medical University, Lublin, 20-090 Lublin, Poland.
²⁰ Department of Rheumatology, Medical University of Lodz, 92-213 Lodz, Poland.
²¹ 2nd Department of Respiratory Medicine, National Tuberculosis and Lung Diseases Research Institute in Warsaw, 01-138 Warsaw, Poland.
²² Department of Allergology, Medical University of Gdańsk, 80-214 Gdansk, Poland.
²³ Patient, not affiliated, 02-673 Warsaw, Poland.

PMID: 36285980
PMCID: PMC9717335
DOI: 10.3390/arm90050052

Abstract

The recommendations were developed as answers to previously formulated questions concerning everyday diagnostic and therapeutic challenges. They were developed based on a review of the current literature using the GRADE methodology. The experts suggest that PF-ILD be diagnosed based on a combination of different criteria, such as the aggravation of symptoms, progression of radiological lesions, and worsening of lung function test parameters. The experts recommend a precise diagnosis of an underlying disease, with serological testing for an autoimmune disease always being included. The final diagnosis should be worked out by a multidisciplinary team (MDT). Patients with an interstitial lung disease other than IPF who do not meet the criteria for the progressive fibrosis phenotype should be monitored for progression, and those with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease. In managing patients with interstitial lung disease associated with autoimmune diseases, an opinion of an MDT should be considered. Nintedanib rather than pirfenidon should be introduced in the event of the ineffectiveness of the therapy recommended for the treatment of the underlying disease, but in some instances, it is possible to start antifibrotic treatment without earlier immunomodulatory therapy. It is also admissible to use immunomodulatory and antifibrotic drugs simultaneously. No recommendations were made for or against termination of anti-fibrotic therapy in the case of noted progression during treatment of a PF-ILD other than IPF. The experts recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied to patients with an interstitial lung disease other than IPF with progressive fibrosis as in patients with IPF.

Keywords: GRADE; PF-ILD; PPF; autoimmune diseases; diagnosis; progressive fibrosing interstitial lung disease; progressive pulmonary fibrosis; treatment.

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Conflict of interest statement

W.J.P. received lecture fees and travel grants and participated in advisory boards for Roche and Boehringer Ingelheim. M.M.M.B. received lecture fees and travel grants from Roche and Boehringer Ingelheim and participated in advisory boards for Boehringer Ingelheim. A.J.B. declared no conflicts of interest. A.B. received travel grants from Boehringer Ingelheim and Roche. B.B. received lecture fees and travel grants and participated in advisory boards for Boehringer Ingelheim. K.B. declared no conflicts of interest. P.W.B. received lecture fees and travel grants from Roche and Boehringer Ingelheim. K.G. received lecture fees and travel grants from Roche and Boehringer Ingelheim and participated in advisory boards for Boehringer Ingelheim. P.G. received lecture fees from Boehringer Ingelheim. E.J. received lecture fees and travel grants and participated in advisory boards for Roche and Boehringer Ingelheim. D.J. received lecture fees and travel grants and participated in advisory boards for Boehringer Ingelheim and Roche. J.K. declared no conflicts of interest. O.K.B. received lecture fees and travel grants from Roche and Boehringer Ingelheim and participated in advisory boards for Boehringer Ingelheim. E.K. received lecture fees and participated in advisory boards for Boehringer Ingelheim. J.K. received lecture fees and travel grants and participated in advisory boards for Roche and Boehringer Ingelheim. B.K.-K. received lecture fees from Roche and Boehringer Ingelheim and travel grants from Boehringer Ingelheim. B.K. received lecture fees and participated in advisory boards for Boehringer Ingelheim. R.L. declared no conflicts of interest. K.L. received lecture fees, travel grants, and research grants and participated in advisory boards for Roche and Boehringer Ingelheim. B.M. received lecture fees and travel grants from Roche and Boehringer Ingelheim and participated in advisory boards for Boehringer Ingelheim. S.M. received lecture fees and travel grants and participated in advisory boards for Roche and Boehringer Ingelheim. J.M. received lecture fees and travel grants from Boehringer Ingelheim. J.M.-D. received lecture fees and travel grants and participated in advisory boards for Boehringer Ingelheim and received travel grants from Roche. E.P. declared no conflicts of interest. A.S. received lecture fees and travel grants from Roche and Boehringer Ingelheim. M.S. (Małgorzata Sobiecka) received lecture fees and travel grants and participated in advisory boards for Roche and Boehringer Ingelheim. R.A.S.-D. declared no conflicts of interest. M.S. (Małgorzata Szołkowska) received lecture fees from Roche and Boehringer Ingelheim. E.W. participated in advisory boards for Boehringer Ingelheim. D.Z. received lecture fees from Roche and Boehringer Ingelheim. P.Ś. received lecture fees and participated in advisory boards for Roche and Boehringer Ingelheim and received travel grants from Boehringer Ingelheim.

References

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Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis

Affiliations

Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis

Authors

Affiliations

Abstract

Conflict of interest statement

References

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources

Medical