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. 2023 Jul 12;12(14):4635.
doi: 10.3390/jcm12144635.

A Real-World Multicenter Retrospective Observational Study on Polish Experience with Nintedanib Therapy in Patients with Idiopathic Pulmonary Fibrosis: The PolExNIB Study

Sebastian Majewski  1 , Adam J Białas  1 , Adam Barczyk  2 , Halina Batura-Gabryel  3 , Małgorzata Buchczyk  4 , Anna Doboszyńska  5 , Katarzyna Górska  6 , Luiza Grabowska-Skudlarz  5 , Hanna Jagielska-Len  7 , Agnieszka Jarzemska  8 , Ewa Jassem  9 , Dariusz Jastrzębski  4 , Aleksander Kania  10 , Marek Koprowski  11 , Michał Krawczyk  12 , Rafał Krenke  6 , Katarzyna Lewandowska  13 , Barbara Mackiewicz  14 , Magdalena M Martusewicz-Boros  15 , Janusz Milanowski  14 , Małgorzata Noceń-Piskorowska  16 , Agata Nowicka  3 , Kazimierz Roszkowski-Śliż  15 , Alicja Siemińska  17 , Krzysztof Sładek  10 , Małgorzata Sobiecka  13 , Tomasz Stachura  10 , Małgorzata Tomczak  18 , Witold Tomkowski  13 , Marzena Trzaska-Sobczak  2 , Dariusz Ziora  4 , Beata Żołnowska  13 , Wojciech J Piotrowski  1
Affiliations

A Real-World Multicenter Retrospective Observational Study on Polish Experience with Nintedanib Therapy in Patients with Idiopathic Pulmonary Fibrosis: The PolExNIB Study

Sebastian Majewski et al. J Clin Med. .

Abstract

Nintedanib is a disease-modifying agent licensed for the treatment of IPF. Data on Polish experience with nintedanib in IPF are lacking. The present study aimed to describe the safety and efficacy profiles of nintedanib in a large real-world cohort of Polish patients with IPF. This was a multicenter, retrospective, observational study of IPF patients treated with nintedanib between March 2018 and October 2021. Data collection included baseline clinical characteristics, results of pulmonary function tests (PFTs), and a six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), and treatment persistence were also retrieved. A total of 501 patients (70% male) with a median age of 70.9 years (IQR 65-75.7) were included in this study. Patients were followed on treatment for a median of 15 months (7-25.5). The majority of patients (66.7%) were treated with the full recommended dose of nintedanib and 33.3% of patients were treated with a reduced dose of a drug. Intermittent dose reductions or drug interruptions were needed in 20% of patients. Over up to 3 years of follow-up, pulmonary function remained largely stable with the minority experiencing disease progression. The most frequent ADRs included diarrhea (45.3%), decreased appetite (29.9%), abdominal discomfort (29.5%), weight loss (32.1%), nausea (20.8%), fatigue (19.2%), increased liver aminotransferases (15.4%), and vomiting (8.2%). A total of 203 patients (40.5%) discontinued nintedanib treatment due to diverse reasons including ADRs (10.2%), death (11.6%), disease progression (4.6%), patient's request (6.6%), and neoplastic disease (2.2%). This real-world study of a large cohort of Polish patients with IPF demonstrates that nintedanib therapy is safe, and is associated with acceptable tolerance and disease stabilization. These data support the findings of previously conducted clinical trials and observational studies on the safety and efficacy profiles of nintedanib in IPF.

Keywords: Poland; efficacy; idiopathic pulmonary fibrosis (IPF); nintedanib; real-world data; safety.

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Conflict of interest statement

S.M. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. A.J.B. declares receiving grants from Boehringer Ingelheim and Roche outside of submitted work. A.B. declares receiving grants and personal fees from Boehringer Ingelheim outside of submitted work. H.B-G. declares no conflict of interest related to this work. M.B. declares receiving personal fees from Boehringer Ingelheim and Roche outside of submitted work. A.D. declares receiving personal fees from Boehringer Ingelheim outside of submitted work. K.G. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. L.G.-S. declares receiving grants and personal fees from Boehringer Ingelheim outside of submitted work. H.J.-L. declares receiving grants and personal fees from Boehringer Ingelheim outside of submitted work. A.J. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. E.J. declares no conflict of interest related to this work. D.J. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. A.K. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. M.K. declares receiving grants and personal fees from Roche outside of submitted work. M.K. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. R.K. declares receiving personal fees from Boehringer Ingelheim outside of submitted work. K.L. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. B.M. declares receiving grants and personal fees from Boehringer Ingelheim outside of submitted work. M.M.M.-B. declares receiving grants and personal fees from Boehringer Ingelheim outside of submitted work. J.M. declares no conflict of interest related to this work. M.N.-P. declares receiving grants and personal fees from Boehringer Ingelheim outside of submitted work. A.N. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. K.R.-Ś. declares no conflict of interest related to this work. A.S. declares receiving personal fees from Boehringer Ingelheim outside of submitted work. KS declares no conflict of interest related to this work. M.S. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. T.S. declares receiving grants and personal fees from Boehringer Ingelheim outside of submitted work. M.T. declares no conflict of interest related to this work. W.T. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. M.T.-S. declares no conflict of interest related to this work. D.Z. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work. B.Ż. declares no conflict of interest related to this work. W.J.P. declares receiving grants and personal fees from Boehringer Ingelheim and Roche outside of submitted work.

Figures

Figure 1
Figure 1
ADRs leading to treatment discontinuation (n = 51). Abbreviations: ADRs—adverse drug reactions, AST—aspartate aminotransferase, ALT—alanine aminotransferase. Dyspepsia includes nausea, vomiting, abdominal discomfort, and decreased appetite.
Figure 2
Figure 2
Changes in FVC% of predicted (FVC%) over the study follow-up period. Change from baseline was calculated as a follow-up timepoint value minus the baseline value; therefore, a negative value indicates a decrease from baseline. Data are presented as median (IQR) values. Abbreviation: FVC—forced vital capacity.
Figure 3
Figure 3
Changes in TLco% of predicted (TLco%) over the study follow-up period. Change from baseline was calculated as a follow-up timepoint value minus the baseline value; therefore, a negative value indicates a decrease from baseline. Data are presented as median (IQR) values. Abbreviation: TLCO—transfer factor of the lung for carbon monoxide.
Figure 4
Figure 4
Changes in 6MWT distance over the study follow-up period. Change from baseline was calculated as a follow-up timepoint value minus the baseline value; therefore, a negative value indicates a decrease from baseline. Data are presented as median (IQR) values. Abbreviation: 6MWT—six-minute walk test.
Figure 5
Figure 5
Longitudinal interval changes in FVC% of predicted (ΔFVC%) during nintedanib treatment. Data are presented as median (IQR) values. Abbreviation: FVC—forced vital capacity.
Figure 6
Figure 6
Longitudinal interval changes in TLCO% of predicted (ΔTLCO%) during nintedanib treatment. Data are presented as median (IQR) values. Abbreviation: TLCO—transfer factor of the lung for carbon monoxide.
Figure 7
Figure 7
The proportion of patients experiencing significant (ΔFVC > 10%) or marginal (10% ≥ ΔFVC > 5%) improvement, stabilization (+5% ≥ ΔFVC > −5%), and marginal (−5% ≥ ΔFVC > −10%) or significant (ΔFVC ≤−10%) decline based on the rate of changes in FVC% of predicted (ΔFVC%) in consecutive 6-month intervals during nintedanib treatment. Abbreviation: FVC—forced vital capacity.
Figure 8
Figure 8
The proportion of patients experiencing significant (ΔTLCO > 15%) improvement, stabilization (+15% ≥ ΔTLCO > −15%), and significant (ΔTLCO ≤ −15%) decline based on the rate of changes in TLCO% of predicted (ΔTLCO%) in consecutive 6-month intervals during nintedanib treatment. Abbreviation: TLCO—transfer factor of the lung for carbon monoxide.
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