Real-world antifibrotic treatment persistence and clinical outcomes for patients with idiopathic pulmonary fibrosis

2021 ◽  
Author(s):  
Ying Qiu ◽  
Julia Zhu ◽  
Brandon Elpers ◽  
Jenny Jiang ◽  
Adesuwa Ogbomo ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Clinical Outcomes ◽  
Real World ◽  
Treatment Persistence

scholarly journals Longitudinal clinical outcomes in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Wim A. Wuyts ◽  
Caroline Dahlqvist ◽  
Hans Slabbynck ◽  
Marc Schlesser ◽  
Natacha Gusbin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Clinical Outcomes ◽  
Real World ◽  
Lung Transplant ◽  
World Population ◽  
Duration Of Treatment

Abstract Background The PROOF registry is an observational study initiated in October 2013 with the aim to monitor disease progression in a real-world population of patients with idiopathic pulmonary fibrosis (IPF). Here, we present longitudinal clinical outcomes from the PROOF registry. Methods Patients with IPF were enrolled across eight centers in Belgium and Luxembourg. For all patients, clinical outcomes data were collected, including mortality, lung transplant, acute exacerbations, and pulmonary hypertension. For patients treated with pirfenidone at any time during follow-up (2013–2017), for any duration of treatment (the pirfenidone-treated population): pirfenidone treatment patterns were collected; changes in pulmonary function (forced vital capacity [FVC] and carbon monoxide diffusing capacity [DLco]) were reviewed up to 24 months post-inclusion; and time-to-event analyses from the time of registry inclusion were performed. Results The PROOF registry enrolled a total of 277 patients. During follow-up, 23.1% of patients died, 5.1% received a lung transplant, 5.4% experienced an acute exacerbation, and 6.1% had comorbid pulmonary hypertension. In the pirfenidone-treated population (N = 233, 84.1%), 12.9% of patients had a temporary dose discontinuation and 31.8% had a temporary dose reduction; 4.3% of patients permanently discontinued pirfenidone due to an adverse drug reaction. Mean percent predicted FVC was 81.2% (standard deviation [SD] 19.0) at Month 0 and 78.3% (SD 25.0) at Month 24, and mean percent predicted DLco was 47.0% (SD 13.2) and 45.0% (SD 16.5), respectively. Rates of ≥ 10% absolute decline in percent predicted FVC and ≥ 15% absolute decline in percent predicted DLco over 24 months were 31.0% and 23.2%, respectively. Mean times from registry inclusion to categorical absolute decline in percent predicted FVC and percent predicted DLco were 20.1 (standard error [SE] 0.6) months and 23.4 (SE 0.5) months, respectively; mean time from registry inclusion to death was 31.0 (SE 0.9) months. Conclusions The PROOF registry is a source of European data characterizing longitudinal clinical outcomes of patients with IPF. Over 12 months of follow-up, pulmonary function remained largely stable in patients with IPF who received pirfenidone for any duration of treatment. Pulmonary function remained similar at 24 months of follow-up, although patient numbers were lower. Trial registration PROOF is registered with the relevant authorities in Belgium and Luxembourg, with registration to Comité National d’Éthique et de Recherche (CNER) N201309/03–12 September 2013 and a notification to Comité National de Protection des Données (CNDP) for Luxembourg.

scholarly journals Turnover of type I and III collagen predicts progression of idiopathic pulmonary fibrosis

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
H. Jessen ◽  
N. Hoyer ◽  
T. S. Prior ◽  
P. Frederiksen ◽  
M. A. Karsdal ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Progression ◽  
Real World ◽  
Progressive Disease ◽  
Type Iii Collagen ◽  
Type I ◽  
Collagen Turnover ◽  
Baseline Levels ◽  
Longitudinal Biomarker

Abstract Background Idiopathic pulmonary fibrosis (IPF) is characterized by the accumulation of fibrillar collagens in the alveolar space resulting in reduced pulmonary function and a high mortality rate. Biomarkers measuring the turnover of type I and III collagen could provide valuable information for prognosis and treatment decisions in IPF. Methods Serological biomarkers reflecting the formation of type III collagen (PRO-C3) and degradation of type I (C1M) and III collagen (C3M) were evaluated in a real-world cohort of 178 newly diagnosed IPF patients. Blood samples and clinical data were collected at baseline, six, and 12 months. Baseline and longitudinal biomarker levels were related to disease progression of IPF (defined as ≥ 5% decline in forced vital capacity (FVC) and/or ≥ 10% decline in diffusing capacity for carbon monoxide (DLco) and/or all-cause mortality at 12 months). Furthermore, we analysed differences in percentage change of biomarker levels from baseline between patients receiving antifibrotic treatment or not. Results Increased baseline levels of type I and III collagen turnover biomarkers were associated with a greater risk of disease progression within 12 months compared to patients with a low baseline type I and III collagen turnover. Patients with progressive disease had higher serum levels of C1M (P = 0.038) and PRO-C3 (P = 0.0022) compared to those with stable disease over one year. There were no differences in biomarker levels between patients receiving pirfenidone, nintedanib, or no antifibrotics. Conclusion Baseline levels of type I and III collagen turnover were associated with disease progression within 12 months in a real-world cohort of IPF patients. Longitudinal biomarker levels of type I and III collagen turnover were related to progressive disease. Moreover, antifibrotic therapy did not affect type I and III collagen turnover biomarkers in these patients. PRO-C3 and C1M may be potential biomarkers for a progressive disease behavior in IPF.

scholarly journals Changes in Neutrophil–Lymphocyte or Platelet–Lymphocyte Ratios and Their Associations with Clinical Outcomes in Idiopathic Pulmonary Fibrosis

2021 ◽  
Vol 10 (7) ◽  
pp. 1427
Author(s):  
Steven D. Nathan ◽  
Jayesh Mehta ◽  
John Stauffer ◽  
Elizabeth Morgenthien ◽  
Ming Yang ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Clinical Outcomes ◽  
Predictive Biomarkers ◽  
Phase Iii ◽  
Response To Treatment ◽  
Reference Ranges ◽  
Neutrophil Lymphocyte Ratio ◽  
Lymphocyte Ratio ◽  
The Relationship

Identification of prognostic and predictive biomarkers in idiopathic pulmonary fibrosis (IPF) could aid assessment of disease severity and prediction of progression and response to treatment. This analysis examined reference ranges for neutrophil–lymphocyte ratio (NLR) and platelet–lymphocyte ratio (PLR) in IPF, and the relationship between NLR or PLR changes and clinical outcomes over 12 months. This post hoc analysis included patients with IPF from the Phase III, double-blind trials of pirfenidone, ASCEND (NCT01366209) and CAPACITY (NCT00287716 and NCT00287729). The relationship between change from baseline to Month 12 in NLR or PLR (divided into quartiles (Q1–Q4)) and outcomes (mortality, respiratory hospitalization, declines in lung function, exercise capacity and quality of life) was assessed. Estimated reference ranges at baseline for all patients analyzed (n = 1334) were 1.1–6.4 for NLR and 56.8–250.5 for PLR. Significant trends were observed across NLR and PLR quartiles for all outcomes in placebo-treated patients, with patients manifesting the greatest NLR or PLR changes experiencing the worst outcomes. These results suggest that the greatest NLR or PLR changes over 12 months were associated with worse clinical outcomes. Further research is needed to determine the utility of NLR and PLR as prognostic biomarkers in IPF.

scholarly journals A retrospective study of the tolerability of nintedanib for severe idiopathic pulmonary fibrosis in the real world

2019 ◽  
Vol 7 (12) ◽  
pp. 262-262 ◽  
Author(s):  
Masayuki Nakamura ◽  
Masaki Okamoto ◽  
Kiminori Fujimoto ◽  
Tomohiro Ebata ◽  
Masaki Tominaga ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Real World ◽  
The Real

Blood mitochondrial DNA as a biomarker of clinical outcomes in idiopathic pulmonary fibrosis

2020 ◽  
Vol 56 (5) ◽  
pp. 2001769
Author(s):  
Hee-young Yoon ◽  
Kwanghun Choi ◽  
Miae Kim ◽  
Hak-Su Kim ◽  
Jin Woo Song
Keyword(s):  
Mitochondrial Dna ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Clinical Outcomes

scholarly journals 4569 Burden of illness in idiopathic pulmonary fibrosis: A real-world cohort

2020 ◽  
Vol 4 (s1) ◽  
pp. 24-24
Author(s):  
Erica Farrand ◽  
Harold Collard
Keyword(s):  
Health Care ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Real World ◽  
Index Date ◽  
Skilled Nursing Facility ◽  
Diagnostic Code ◽  
Nursing Facility ◽  
Skilled Nursing ◽  
Outpatient Visits

OBJECTIVES/GOALS: Studying IPF associated health care utilization (HRU) in real world settings, provides the opportunity to produce generalizable results that can directly inform models of care delivery. The objective of this study was to examine real-world differences in the natural history of annual HRU and treatment trends associated with IPF in a large, community-based population of patients with IPF, compared to matched non-IPF controls. METHODS/STUDY POPULATION: Cases of IPF were identified using case validated algorithms in the Kaiser Permanente Northern California adult population from 2000 to 2014. Each case was matched to at least one and no more than five non-IPF controls by age, sex, race/ethnicity and length of enrollment. The date of the first occurrence of the IPF-specific diagnostic code was considered the index date for cases and matched controls. Comorbidity burden and HRU was assessed in the five years pre- and post-index date, including hospitalizations, outpatient visits, use of diagnostic and monitoring studies and medications. Poisson generalized estimating equations models with robust standard errors were used to estimate adjusted case-control differences in HRU, accounting for clustering within matched sets. RESULTS/ANTICIPATED RESULTS: 691 patients were identified with incident IPF during the study period and matched to 3,452 control subjects. Demographics were well balanced between cases and controls due to matching. Patients with IPF had a higher burden of all selected co-morbidities and higher HRU compared to controls. In the pre-index period, IPF members had significantly higher rates of all diagnostic procedures (p < 0.001) and health care visits, with the exception of skilled nursing facility care (p < 0.001). The greatest relative difference was observed with use of Chest CT (RR = 245.94, 95% CI 117.04, 516.82). In the post-index period compared to controls, patients with IPF had higher rates of serial testing (p < 0.001) and inpatient and outpatient care including, all-cause hospitalization (1.55), emergency room visits (1.19), outpatient visits (1.18), and skilled nursing facility stay (1.35). DISCUSSION/SIGNIFICANCE OF IMPACT: Patients with idiopathic pulmonary fibrosis experience increased co-morbidity and healthcare resource utilization compared to controls. This increased burden extends beyond the index-period as previously documented and is true for a large real-world cohort. CONFLICT OF INTEREST DESCRIPTION: NA

Sign in / Sign up

Export Citation Format

Share Document