A multicentre retrospective observational study on Polish experience of pirfenidone therapy in patients with idiopathic pulmonary fibrosis: the PolExPIR study

Majewski, Sebastian; Białas, Adam J.; Buchczyk, Małgorzata; Gomółka, Paweł; Górska, Katarzyna; Jagielska-Len, Hanna; Jarzemska, Agnieszka; Jassem, Ewa; Jastrzębski, Dariusz; Kania, Aleksander; Koprowski, Marek; Krenke, Rafał; Kuś, Jan; Lewandowska, Katarzyna; Martusewicz-Boros, Magdalena; Roszkowski‐Śliż, Kazimierz; Siemińska, Alicja; Sładek, Krzysztof; Sobiecka, Małgorzata; Szewczyk, Karolina; Tomczak, Małgorzata; Tomkowski, Witold; Wiatr, Elżbieta; Zolnowska, Beata; Piotrowski, Wojciech J.

doi:10.1186/s12890-020-1162-6

Cited by 11 publications

(16 citation statements)

References 40 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In our series, the median increase in FVC was 2.7% of predicted, and a decrease in TLCO was 0.8% of predicted (data not shown) and none of the study participants were excluded from further analysis because of a lung function decline surpassing the threshold of 10% of baseline FVC. Of note, we found a significant decrease in BMI after six months of treatment with pirfenidone, which is consistent with earlier findings on the adverse effects of this drug [39,40]. Despite the lack of differences in EBC VEGF-A between IPF patients and controls and between IPF patients at baseline and after six months of treatment with pirfenidone, our findings provide some new data supporting the usefulness of VEGF-A as a marker in IPF.…”

Section: Discussionsupporting

confidence: 92%

“…In the study by Yoshikawa et al, an FVC decline of ≥10% and/or a TLCO decline of ≥15% of baseline was observed in 34.7% of the investigated group after six to nine months of observation [38]. Majewski et al reported a more favorable treatment response, with FVC and TLCO stability in 60.8% and 87.1% of the patients over six months of treatment with pirfenidone, respectively [39]. In our series, the median increase in FVC was 2.7% of predicted, and a decrease in TLCO was 0.8% of predicted (data not shown) and none of the study participants were excluded from further analysis because of a lung function decline surpassing the threshold of 10% of baseline FVC.…”

Section: Discussionmentioning

confidence: 96%

See 1 more Smart Citation

Exhaled Biomarkers in Idiopathic Pulmonary Fibrosis—A Six-Month Follow-up Study in Patients Treated with Pirfenidone

Jaśkiewicz

Mycroft

Maskey-Warzęchowska

et al. 2020

JCM

View full text Add to dashboard Cite

The mechanism of action of pirfenidone in idiopathic pulmonary fibrosis (IPF) has not been fully elucidated. To offer additional insight, we evaluated the change in the cytokine profile in exhaled breath condensate (EBC) following a six-month treatment with pirfenidone in patients with IPF. EBC concentrations of interleukin (IL)-6, IL-8, IL-15, TNF-α and VEGF-A were assessed with ELISA and compared at baseline and after six months of pirfenidone treatment. Twenty-nine patients with IPF and 13 controls were evaluated at baseline. With the exception of IL-8 concentration, which was lower in patients with IPF when compared to controls (p = 0.005), the cytokine levels did not differ between the groups. Despite the use of a high sensitivity assay, IL-8 reached detectable values only in 24% of IPF patients. EBC analysis after six months of treatment with pirfenidone did not reveal any differences in the cytokine levels. The change in EBC vascular endothelial growth factor A (VEGF-A) correlated with the change in the 6 min walk distance (r = 0.54, p = 0.045). We conclude that a six-month treatment with pirfenidone did not significantly change the EBC cytokine profile. Our findings support the potential usefulness of VEGF-A as a marker in IPF. The low EBC IL-8 level in patients with IPF is a novel finding which needs confirmation in larger studies.

show abstract

Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 96%

Exhaled Biomarkers in Idiopathic Pulmonary Fibrosis—A Six-Month Follow-up Study in Patients Treated with Pirfenidone

Jaśkiewicz

Mycroft

Maskey-Warzęchowska

et al. 2020

JCM

View full text Add to dashboard Cite

show abstract

“…Second, participants were highly motivated and deemed taking medication important, confirming available evidence [ 25 ]. Only 3% of our participants reported having discontinued pirfenidone based on their own initiative, which is slightly lower than other real-world studies, reporting a 5.5–6% discontinuation rate of pirfenidone [ 26 , 27 ]. However, we detected problems with adherence already early after treatment initiation (19.6% at week six), and nonadherence increased over time (up to 36.4% at year two).…”

Section: Discussioncontrasting

confidence: 63%

Behavioural and psychological patterns of patients with idiopathic pulmonary fibrosis: a prospective study

et al. 2022

View full text Add to dashboard Cite

Background Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung condition. Currently, care models predominantly focus on acute medical and pharmacological needs. As a step towards holistic care, the aim of this prospective study was to investigate the psychological and behavioural needs of IPF patients treated with pirfenidone from diagnosis until two years of follow-up. Methods The following variables were selected from the literature on patients’ needs and the COM-B model, a theoretical model explaining behaviour: medication adherence, barriers to adherence, importance and intentions of medication adherence, anxiety, depression, health literacy, knowledge, reported side effects, adherence to sun protection recommendations, alcohol use, physical activity, quality of life and health status. Linear and generalised linear models for longitudinal data were used to evaluate the evolution since treatment initiation. Results We included 66 outpatients: 72.7% men, mean age of 70.3 years (range 50–87), predicted mean forced vital capacity of 85.8% (SD 17.4) and predicted mean diffusing capacity for monoxide of 56.9% (SD 15.7). The participants placed considerable importance on following the treatment recommendations. We noticed difficulties regarding health literacy, alcohol use, pirfenidone adherence (decline over time) and adherence to sun protection recommendations (early in follow-up care). There were low levels of physical activity (no effect of time), high body mass indices (decline over time) and moderate levels of depression and anxiety. Conclusion When providing care to IPF patients, behavioural issues, health literacy and psychological well-being should be taken into consideration. There is a need to further explore interventions and care models to tackle these difficulties. Trial registration This study was registered in the ClinicalTrials.gov database (identifier NCT03567785) on May 9th, 2018

show abstract

“…The authors presumed that efficacy of pirfenidone in IPF patients with severe lung function impairment may diminish after 6 months of treatment [ 27 ]. In a multicenter retrospective IPF study, Majewski et al found that the median annual decline in FVC during the first year of pirfenidone therapy was −20 mL and during the second year was −120 mL ( n = 307); this decline was even less than that noted in the ASCEND trial, in which mean decline from baseline in FVC was −235 mL in the pirfenidone group over 52 weeks [ 21 , 28 ]. Similarly, in another real-world study focusing on long-term use of pirfenidone in the large cohort ( n = 502) of Japanese IPF patients, Bando et al reported a mean decline of FVC of −30 mL and of −158 mL in the first and second year of therapy, respectively [ 29 ].…”

Section: Discussionmentioning

confidence: 99%

Assessing the Effectiveness of Pirfenidone in Idiopathic Pulmonary Fibrosis: Long-Term, Real-World Data from European IPF Registry (eurIPFreg)

Krauss

Tello

Wilhelm

et al. 2020

JCM

View full text Add to dashboard Cite

Background: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrotic pulmonary disease with rising incidence. In this study the effectiveness of pirfenidone, as measured by longitudinal change in individual slope of forced vital capacity (FVC) prior to and after initiating pirfenidone treatment, was evaluated in IPF patients recruited into the European registry for idiopathic pulmonary fibrosis (eurIPFreg). Secondary variables were the evaluation of the change in individual slope of diffusion capacity of the lungs for carbon monoxide (DLco), the Borg dyspnea scale, and six-minute walking distance (6MWD), as well as survival analyses. Results: Data of 122 eurIPFreg patients, who had at least two pulmonary function tests (PFTs) prior to or under treatment with pirfenidone, were analyzed by calculating slope-changes. The global analysis revealed an average slope change of +1.48 ± 0.28 (% per annum (p.a)) after start of treatment (p < 0.001), reflecting a reduction in annual FVC decline of approx. 50% under pirfenidone; it also showed a reduction in DLco, and increase in 6MWD (both p < 0.0001), as well as a flattening of the Borg dyspnea scale (p = 0.02). The median survival under treatment was 4.82 years. Patients with a more restrictive disease (FVC < 80% pred.), with a rapid progression (FVC decline >10% pred. p.a.), previous smokers and patients > 60 years of age seemed to profit more from pirfenidone treatment. Conclusions: We report the effectiveness of pirfenidone in a European “real world” IPF cohort with outcome data extending up to 9 years. Global analyses demonstrated a positive effect of pirfenidone on the decline of the lung function over time. Survival was dependent on Gender–Age–Physiology (GAP) score and age prior to therapy.

show abstract

A multicentre retrospective observational study on Polish experience of pirfenidone therapy in patients with idiopathic pulmonary fibrosis: the PolExPIR study

Cited by 11 publications

References 40 publications

Exhaled Biomarkers in Idiopathic Pulmonary Fibrosis—A Six-Month Follow-up Study in Patients Treated with Pirfenidone

Exhaled Biomarkers in Idiopathic Pulmonary Fibrosis—A Six-Month Follow-up Study in Patients Treated with Pirfenidone

Behavioural and psychological patterns of patients with idiopathic pulmonary fibrosis: a prospective study

Assessing the Effectiveness of Pirfenidone in Idiopathic Pulmonary Fibrosis: Long-Term, Real-World Data from European IPF Registry (eurIPFreg)

Contact Info

Product

Resources

About