Real World Experiences: Pirfenidone and Nintedanib are Effective and Well Tolerated Treatments for Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic Pulmonary Fibrosis (IPF) now has two licensed treatments available. Pirfenidone was the first drug to be licensed and approved for use, followed by nintedanib. We set out our real world experience with these agents in terms of their adverse events profile outside the restrictions of a clinical trial. We have demonstrated in the real world setting, that side effects are common and predominantly gastrointestinal with both therapies. Our study shows that the side effects can be effectively managed in the majority of patients with an acceptable discontinuation rate similar to that seen in the clinical trials. These findings are compelling despite the fact that the patients in our study are older, have severer disease as depicted by baseline lung function and more co-morbidities. Our data provides ongoing evidence of the safety and tolerability of both pirfenidone and nintedanib in patients who would not have met the rigorous criteria to be included in a clinical trial. Both these agents are effective in the management of IPF and slow the progression of this debilitating life limiting condition.

Real-World Experience with Nintedanib in Patients with Idiopathic Pulmonary Fibrosis

“…The demographic characteristics of the patients reported in this study, including age, sex distribution, and smoking history, were broadly similar to those reported in the previous INPULSIS trials [11] as well as to those reported in other real-world reports [15, 16], reflecting the typical patient demographic characteristics of patients with IPF. Subgroup analyses of the INPULSIS trials have shown that the treatment effects of nintedanib are consistent across patient subgroups, including sex, age, smoking status, and baseline respiratory functions measured using baseline percent predicted FVC [26].…”

“…As such, our data provide support for the benefits of nintedanib in a patient population with more severe disease at baseline; nintedanib treatment was associated with stable disease at 6 months, as measured by FVC, in the majority (67%) of patients. The baseline respiratory function reported in our real-world cohort was similar to that in patients treated in other real-world analyses [15, 16]. …”

“…Results recently published in abstract form from the ongoing INPULSIS®-ON extension study have confirmed the long-term benefit of nintedanib in reducing disease progression and the long-term safety and tolerability profile of nintedanib in patients suffering from IPF [14]. Only minimal data of patients treated in the real-world setting have been published so far and include data from a cohort of 62 patients treated with nintedanib as part of the German compassionate use programme with a mean treatment duration 14 months [15] and data from a cohort of 124 patients treated with nintedanib in the UK in which the average length of treatment was around 9 months [16]. These findings confirmed the results of controlled trials.…”

Pirfenidone safety and adverse event management in idiopathic pulmonary fibrosis

“…In a single-centre, retrospective, observational study of 351 patients who were receiving pirfenidone, 75% of reported AEs were GI-related, with loss of appetite (17%) and nausea/vomiting (15%) being most frequent, similar to what was observed in the phase III trials [10,11,33]. The highest number of treatment discontinuations occurred with appetite loss and nausea/vomiting (28 and 22, respectively).…”

Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age

“…Nintedanib is therefore contra-indicated in patients with significant active cardiovascular disease and those taking dual anti-platelet therapy or warfarin. Post-trial and realworld data has not demonstrated any additional safety signals with respect to cardiovascular and bleeding risks (16,21). Nintedanib is contraindicated in patients with peanut allergy.…”