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

Krauss, Ekaterina; Tello, Silke; Wilhelm, Jochen; Schmidt, Jan Peter; Stoehr, Mark; Seeger, Werner; Dartsch, Ruth C.; Crestani, Bruno; Güenther, Andreas

doi:10.3390/jcm9113763

Cited by 17 publications

(11 citation statements)

References 36 publications

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“…Because IPF is a heterogeneous disease with a variable course, predicting disease outcomes is difficult. Indices of lung function at both baseline and functional decline in the progression of the disease have proved to have prognostic significance in IPF; that is, lower FVC and DLCO at baseline herald greater decline in lung function and poor prognosis (20,21). In accordance with that, our present study found that the baseline FVC and DLCO were lower in the non-survival group.…”

Section: Discussionsupporting

confidence: 88%

Soluble ST2 in serum predicts the prognosis of idiopathic pulmonary fibrosis: a retrospective study

Yu¹,

Gui²,

Yu³

et al. 2022

Ann Transl Med

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Background: Idiopathic pulmonary fibrosis (IPF) is a heterogeneous and progressive fibrosing interstitial lung disease with a poor prognosis. However, there are currently no effective biomarker that can reliably predict the prognosis for IPF in clinic. The serum level of soluble suppression of tumorigenicity-2 (sST2), which is involved in the immune response, has proven to be a prognostic predictor for various diseases.Previous studies have confirmed that the immune dysfunction plays an important role in the pathogenesis of IPF and the serum sST2 concentrations in patients with IPF are elevated. However, the relationship between sST2 and the prognosis of IPF remains unknown.Methods: A total of 83 patients with IPF and 20 healthy controls from 2016 to 2021 were enrolled and demographic variables, indices of lung function testing as well as the biomarkers including the sST2 were obtained at baseline. During follow-up, the primary endpoint was defined as all-cause death and clinical deterioration. Cox hazard models and Kaplan-Meier method were used to assess the prognostic value of various indices including sST2.Results: Mean duration of follow-up was 29 months, during which 49 patients had an event, and of them, 35 patients died. The sST2 level was higher in the IPF patients compared with the healthy controls.Although the sST2 level did not directly predict all-cause death in the present study, it was proved to be an independent predictor of event-free survival. Multivariate forward stepwise model which was adjusted by age, sex, and body surface area (BSA) showed that the overexpression of sST2 increased the hazard ratio [1.005, 95% confidence interval (CI): 1.001-1.010]. A higher sST2 serum level heralded more deterioration and the poor outcomes. Moreover, the effect of sST2 on the prognosis of IPF may not necessarily involve the development of IPF-related pulmonary hypertension (PH).Conclusions: In our study, the sST2 serum level was significantly elevated and a higher serum level of sST2 predicted more deterioration and poor outcomes in patients with IPF. Thus, sST2 can serve as a valuable prognostic biomarker for the outcome of IPF. However, further multicenter clinical trials of larger sample size are needed in the future.

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Section: Discussionsupporting

confidence: 88%

Soluble ST2 in serum predicts the prognosis of idiopathic pulmonary fibrosis: a retrospective study

Yu¹,

Gui²,

Yu³

et al. 2022

Ann Transl Med

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“…Our cohort, together with other two PPF cohorts, didn't reach the media survival time with 4-year, 5-year and 7-year follow up respectively. In contrast, the media survival time of IPF is 3-5 years according to previous reports [14,20]. These data suggest that the change in FVC% was similar in patients with IPF and patients of PPF, but the overall survival of PPF was better than that of IPF.…”

Section: Discussionsupporting

confidence: 61%

Compare three diagnostic criteria of progressive pulmonary fibrosis

Chen

Chunfang

2022

Preprint

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Background and objective: In patients with fibrotic interstitial lung disease (ILD), a progressive fibrosing phenotype (PPF) typically demonstrates worsening respiratory symptoms, lung function decline and continuing fibrosis. The goal of this study was to compare the three different diagnostic criteria of PPF. Methods: Except for idiopathic pulmonary fibrosis, all consecutive adult patients with fibrotic ILD were retrospectively examined for the three predefined diagnostic criteria of PPF. The three criteria assess the disease progression in preceding 6 (half-year), 12 (one-year) and 24 (two-year) months respectively. The clinical characteristics, decline in predicted percent of forced vital capacity (FVC%) and survival of the three groups were compared, followed by determination of risk factors for mortality. Results: We identified 246 patients by half-year standard, 154 patients by one-year standard and 281 patients by two-year standard. Among them, 95% of patients in one-year group were also included in two-year group. The average one-year decline in FVC% was -1.0%, -2.7%, and -4.1% for half-year, one-year and two-year group respectively. The 4-year survival rate was 74% in half-year group, 66% in one-year group and 62% in two-year group. In multivariate Cox model, only baseline DLCO%<50% was correlated with mortality, with a hazard ratio of 3.4 (95% CI 1.1-10.6, P=0.03). Conclusion: In the current situations, both the one-year and two-year criterion are the reasonable choice to define PPF both in researches and clinical practice, and DLCO% is an independent predictor for mortality of PPF.

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“…The antifibrotic drug, pirfenidone, reduces lung function decline in patients with IPF compared with placebo [4,5] and is used in clinical practice [6,7]. In addition, several recent studies have reported that pirfenidone might reduce mortality in patients with IPF [8,9].…”

Section: Introductionmentioning

confidence: 99%

Factors associated with dose reduction of pirfenidone in patients with idiopathic pulmonary fibrosis: A study based on real-world clinical data

et al. 2023

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Introduction Although pirfenidone slows disease progression in patients with idiopathic pulmonary fibrosis (IPF), in clinical practice, patients often cannot tolerate the recommended dose because of several adverse events. This study aimed to investigate adverse events associated with pirfenidone and factors associated with dose reduction. Methods This single-center retrospective cohort study included 156 consecutive patients with IPF who received pirfenidone. Demographic characteristics, pulmonary function, and pirfenidone-related adverse events were investigated. We compared patients who received standard and reduced doses of pirfenidone. Results The mean patient age was 69.7 years. The median follow-up duration was 243 days. The low-dose group (n = 73) included older patients (71.0 years vs. 67.4 years, p = 0.016), fewer smokers (80.8% vs. 96.4%, p = 0.008), and patients with a lower body mass index (BMI; 24.1 kg/m2 vs. 25.7 kg/m2, p = 0.027) than the standard dose group (n = 57). Multivariate logistic regression analysis revealed that older age (odds ratio = 1.066, p = 0.016) was significantly associated with dose reduction of pirfenidone after adjusting for sex, smoking history, emphysema, and BMI. No significant difference was found in the rates of a reduced forced vital capacity and diffusing capacity for carbon monoxide between the two groups. Conclusions Although older patients are more likely to undergo dose reduction of pirfenidone, low-dose pirfenidone might be effective for treating patients with IPF. Low-dose pirfenidone could be considered an effective treatment option for older patients with IPF.

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

Cited by 17 publications

References 36 publications

Soluble ST2 in serum predicts the prognosis of idiopathic pulmonary fibrosis: a retrospective study

Soluble ST2 in serum predicts the prognosis of idiopathic pulmonary fibrosis: a retrospective study

Compare three diagnostic criteria of progressive pulmonary fibrosis

Factors associated with dose reduction of pirfenidone in patients with idiopathic pulmonary fibrosis: A study based on real-world clinical data

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