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 Pirfenidone safety and adverse event management in idiopathic pulmonary fibrosis

2017 ◽  
Vol 26 (146) ◽  
pp. 170057 ◽  
Author(s):  
Lisa H. Lancaster ◽  
Joao A. de Andrade ◽  
Joseph D. Zibrak ◽  
Maria L. Padilla ◽  
Carlo Albera ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Real World ◽  
Progression Free Survival ◽  
Controlled Clinical Trials ◽  
Lung Function Decline ◽  
Real World Data ◽  
Similar Treatment ◽  
Randomised Controlled

Pirfenidone is one of two approved therapies for the treatment of idiopathic pulmonary fibrosis (IPF). Randomised controlled clinical trials and subsequent post hoc analyses have demonstrated that pirfenidone reduces lung function decline, decreases mortality and improves progression-free survival. Long-term extension trials, registries and real-world studies have also shown similar treatment effects with pirfenidone. However, for patients with IPF to obtain the maximum benefits of pirfenidone treatment, the potential adverse events (AEs) associated with pirfenidone need to be managed. This review highlights the well-known and established safety profile of pirfenidone based on randomised controlled clinical trials and real-world data. Key strategies for preventing and managing the most common pirfenidone-related AEs are described, with the goal of maximising adherence to pirfenidone with minimal AEs.

scholarly journals Long-term treatment of patients with idiopathic pulmonary fibrosis with nintedanib: results from the TOMORROW trial and its open-label extension

Thorax ◽  
2017 ◽  
Vol 73 (6) ◽  
pp. 581-583 ◽  
Author(s):  
Luca Richeldi ◽  
Michael Kreuter ◽  
Moisés Selman ◽  
Bruno Crestani ◽  
Anne-Marie Kirsten ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Term Treatment ◽  
Adverse Event Profile ◽  
Open Label ◽  
Comparator Group ◽  
Long Term Treatment ◽  
Open Label Extension ◽  
Rate Of Decline

The TOMORROW trial of nintedanib comprised a randomised, placebo-controlled, 52-week period followed by a further blinded treatment period and an open-label extension. We assessed outcomes across these periods in patients randomised to nintedanib 150 mg twice daily or placebo at the start of TOMORROW. The annual rate of decline in FVC was −125.4 mL/year (95% CI −168.1 to −82.7) in the nintedanib group and −189.7 mL/year (95% CI −229.8 to −149.6) in the comparator group. The adverse event profile of nintedanib remained consistent throughout the studies. These results support a benefit of nintedanib on slowing progression of idiopathic pulmonary fibrosis beyond 52 weeks.

scholarly journals Long‐term treatment with nintedanib in Asian patients with idiopathic pulmonary fibrosis: Results from INPULSIS®‐ON

Respirology ◽  
2019 ◽  
Vol 25 (4) ◽  
pp. 410-416 ◽  
Author(s):  
Jin Woo Song ◽  
Takashi Ogura ◽  
Yoshikazu Inoue ◽  
Zuojun Xu ◽  
Manuel Quaresma ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Term Treatment ◽  
Asian Patients ◽  
Long Term Treatment

scholarly journals Real-World Practice Patterns for Prevention and Management of Potential Adverse Events with Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis

2018 ◽  
Vol 4 (1) ◽  
pp. 103-114 ◽  
Author(s):  
Mark L. Wencel ◽  
Tmirah Haselkorn ◽  
Susan L. Limb ◽  
John L. Stauffer ◽  
Elizabeth Morgenthien ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Adverse Events ◽  
Real World ◽  
Practice Patterns

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.

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