scholarly journals A real world retrospective observational study exploring the effectiveness and safety of antifibrotics in idiopathic pulmonary fibrosis

2020 ◽  
Author(s):  
William Alexander Wright ◽  
Louise Crowley ◽  
Dhruv Parekh ◽  
Anjali Crawshaw ◽  
Davinder Dosanjh ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Observational Study ◽  
Real World ◽  
Progression Free Survival ◽  
Retrospective Observational Study ◽  
Control Group ◽  
Antifibrotic Therapy ◽  
Drug Tolerability ◽  
Pre Treatment

Abstract Background Pirfenidone and nintedanib are the only disease-modifying treatments available for idiopathic pulmonary fibrosis (IPF). Our aim was to test their effectiveness and safety in clinical practice.Methods This is a single centre retrospective observational study undertaken at a specialised interstitial lung disease centre in England. Data including progression-free survival (PFS), mortality and drug tolerability were compared between IPF patients on antifibrotic therapies and an untreated control group who had a forced vital capacity percentage (FVC %) predicted within the licensed antifibrotic treatment range.Results 104 patients received antifibrotic therapies and 64 control patients were identified. PFS at 6 months was significantly greater in the antifibrotic group (75.0%) compared to the control group (56.3%) (p=0.012). PFS was not significant at 12 or 18 months. The 12-month post-treatment mean decline in FVC% predicted (-4.6±6.2%) was significantly less than the 12-month pre-treatment decline (-10.4 ±11.8%) (p=0.039). The 12-month mortality rate was not significantly different between the antifibrotic group (25.3%) and the control group (35.5%) (p=0.132). Baseline BMI≤25, baseline DLco% predicted ≤35 and antifibrotic discontinuation within 3 months were independent predictors of 12-month mortality. Antifibrotic discontinuation was significantly higher by 3 and 6 months for patients on pirfenidone than those on nintedanib (p=0.006 and p=0.044, respectively). Discontinuation at 12 months was not significantly different (p=0.381).Conclusion This real-world study revealed that antifibrotics are having promising effects on PFS, lung function and mortality. These findings may favour commencement of nintedanib as first line antifibrotic therapy, given the lower rates of early treatment discontinuation.

scholarly journals Real-world retrospective observational study exploring the effectiveness and safety of antifibrotics in idiopathic pulmonary fibrosis

2021 ◽  
Vol 8 (1) ◽  
pp. e000782
Author(s):  
William Alexander Wright ◽  
Louise E Crowley ◽  
Dhruv Parekh ◽  
Anjali Crawshaw ◽  
Davinder P Dosanjh ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Observational Study ◽  
Real World ◽  
Progression Free Survival ◽  
Retrospective Observational Study ◽  
Control Group ◽  
Baseline Body Mass Index ◽  
Antifibrotic Therapy ◽  
Drug Tolerability

BackgroundPirfenidone and nintedanib are the only disease-modifying treatments available for idiopathic pulmonary fibrosis (IPF). Our aim was to test their effectiveness and safety in clinical practice.MethodsThis is a single-centre retrospective observational study undertaken at a specialised interstitial lung disease centre in England. Data including progression-free survival (PFS), mortality and drug tolerability were compared between patients with IPF on antifibrotic therapies and an untreated control group who had a forced vital capacity percentage (FVC %) predicted within the licensed antifibrotic treatment range.Results104 patients received antifibrotic therapies and 64 control patients were identified. PFS at 6 months was significantly greater in the antifibrotic group (75.0%) compared with the control group (56.3%) (p=0.012). PFS was not significant at 12 or 18 months when comparing the antifibrotic group with the control group. The 12-month post-treatment mean decline in FVC % predicted (−4.6±6.2%) was significantly less than the 12-month pretreatment decline (−10.4±11.8%) (p=0.039). The 12-month mortality rate was not significantly different between the antifibrotic group (25.3%) and the control group (35.5%) (p=0.132). Baseline Body Mass Index of≤25, baseline diffusion capacity for carbon monoxide percentage predicted of ≤35 and antifibrotic discontinuation within 3 months were independent predictors of 12-month mortality. Antifibrotic discontinuation was significantly higher by 3 and 6 months for patients on pirfenidone than those on nintedanib (p=0.006 and p=0.044, respectively). Discontinuation at 12 months was not significantly different (p=0.381).ConclusionsThis real-world study revealed that antifibrotics are having promising effects on PFS, lung function and mortality. These findings may favour commencement of nintedanib as first-line antifibrotic therapy, given the lower rates of early treatment discontinuation, although further studies are required to investigate this.

scholarly journals Long-term clinical and real-world experience with pirfenidone in the treatment of idiopathic pulmonary fibrosis

2015 ◽  
Vol 24 (135) ◽  
pp. 58-64 ◽  
Author(s):  
Vincent Cottin ◽  
Toby Maher
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Adverse Events ◽  
Real World ◽  
Sun Exposure ◽  
Progression Free Survival ◽  
Primary Treatment ◽  
Lung Function Decline ◽  
Long Term Treatment

Idiopathic pulmonary fibrosis (IPF) is a chronic, irreversible, progressively destructive lung disease that culminates in respiratory failure and death. Randomised controlled trials have demonstrated that treatment of IPF patients with pirfenidone reduces lung function decline, improves progression-free survival and significantly reduces the risk of all-cause mortality at 1 year. Pirfenidone has been shown to have a favourable safety profile and was generally well tolerated over the long term in clinical trials and real-world experience. However, side-effect management is critical to help some patients remain on treatment over the long term. The primary treatment-related adverse events associated with pirfenidone therapy are gastrointestinal upset, rash and photosensitivity. Gastrointestinal events may be mitigated by ensuring that pirfenidone is taken with food, while skin symptoms may be reduced by avoiding sun exposure and frequent use of sunblock. Educating patients about the potential for these adverse events to occur and providing instructions prior to treatment to avoid adverse drug reactions are an important means of ensuring patients may derive the important benefits provided by long-term treatment with pirfenidone.

scholarly journals Retrospective observational study of trends in hospital admissions for idiopathic pulmonary fibrosis in Spain (2004–2013) using administrative data

BMJ Open ◽  
2017 ◽  
Vol 7 (2) ◽  
pp. e013156 ◽  
Author(s):  
Fernando Pedraza-Serrano ◽  
Ana López de Andrés ◽  
Rodrigo Jiménez-García ◽  
Isabel Jiménez-Trujillo ◽  
Valentín Hernández-Barrera ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Observational Study ◽  
Administrative Data ◽  
Hospital Admissions ◽  
Retrospective Observational Study

Antithrombotics in idiopathic pulmonary fibrosis patients with concomitant antifibrotic therapy: a single centre real world experience

2020 ◽  
Author(s):  
Anna Agnese Stanziola ◽  
Danilo Pentangelo ◽  
Rossella D'Angelo ◽  
Ludovica Capitelli ◽  
Roberto Rega ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Real World ◽  
Single Centre ◽  
Antifibrotic Therapy

scholarly journals Anticoagulant Use and Bleeding Risk in Central European Patients with Idiopathic Pulmonary Fibrosis (IPF) Treated with Antifibrotic Therapy: Real-World Data from EMPIRE

Drug Safety ◽  
2020 ◽  
Vol 43 (10) ◽  
pp. 971-980 ◽  
Author(s):  
Abigél M. Kolonics-Farkas ◽  
Martina Šterclová ◽  
Nesrin Mogulkoc ◽  
Jan Kus ◽  
Marta Hájková ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Real World ◽  
Bleeding Risk ◽  
Real World Data ◽  
Central European ◽  
World Data ◽  
Antifibrotic Therapy

scholarly journals Genetic Variation in CCL18 Gene Influences CCL18 Expression and Correlates with Survival in Idiopathic Pulmonary Fibrosis—Part B

2020 ◽  
Vol 9 (6) ◽  
pp. 1993
Author(s):  
Canay Caliskan ◽  
Benjamin Seeliger ◽  
Benedikt Jäger ◽  
Jan Fuge ◽  
Tobias Welte ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Progressive Disease ◽  
Progression Free Survival ◽  
Serum Levels ◽  
Enzyme Linked Immunosorbent Assay ◽  
Free Survival ◽  
Antifibrotic Therapy ◽  
Level Measurement ◽  
Chemokine Ligand

Idiopathic pulmonary fibrosis (IPF) is a progressive disease with high mortality. CC-chemokine ligand 18 (CCL18) is predictive of survival in IPF. We described correlation of CCL18 serum levels with the genotype of rs2015086 C > T polymorphism the CCL18-gene, which was associated with survival in a pre-antifibrotic cohort (Part-A). Herein (Part-B), we aimed to validate these findings and to study the effects of antifibrotics. Two cohorts were prospectively recruited, cohort-A (n = 61, pre-antifibrotic) and cohort B (n = 101, received antifibrotics). Baseline CCL18 serum level measurement by enzyme-linked immunosorbent assay (ELISA, serially in cohort B) and genotyping of rs2015086 was performed and correlated with clinical outcomes. The CT genotype was present in 15% and 31% of patients. These patients had higher CCL18 levels compared to the TT-genotype (cohort-A: 234 vs. 115.8 ng/mL, p < 0.001; cohort B: 159.5 vs. 120 ng/mL, p = 0.0001). During antifibrotic therapy, CCL18 increased (p = 0.0036) regardless of rs2015086-genotype and antifibrotic-agent. In cohort-A, baseline CCL18-cutoff (>120 ng/mL) and CT-genotype were associated with mortality (p = 0.041 and p = 0.0051). In cohort-B, the CCL18-cutoff (>140 ng/mL) was associated with mortality (p = 0.003) and progression (p = 0.004), but not the CT/CC-genotype. In conclusion, we validated the correlation between rs2015086-genotype and CCL18 serum levels, which was predictive of (progression-free)-survival in two prospective validation cohorts.

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