Abstract
The discovery of antifibrotic agents have resulted in advances in the therapeutic management of idiopathic pulmonary fibrosis (IPF). Currently, nintedanib and pirfenidone have become the basis of IPF therapy based on the results of large randomized clinical trials showing their safety and efficacy in reducing disease advancement. However, the goal of completely halting disease progress has not been reached yet. Administering nintedanib with add-on pirfenidone is supposed to enhance the therapeutic benefit by simultaneously acting on two different pathogenic pathways. All this becomes more important in the context of the ongoing global pandemic of coronavirus disease 2019 (COVID-19) because of the fibrotic consequences following SARS-CoV-2 infection in some patients. However, little information is available about their drug–drug interaction, which is important mainly in polymedicated patients. The aim of this review is to describe the current management of progressive fibrosing interstitial lung diseases (PF-ILDs) in general and of IPF in particular, focusing on the pharmacokinetic drug-drug interactions between these two drugs and their relationship with other medications in patients with IPF.
Highlights
Interstitial lung diseases (ILD) are a heterogeneous group of pulmonary disorders characterized by varying degrees of inflammation and fibrosis resulting in the loss of alveolar function and impairment of gas exchange [1]
In the United Kingdom, Idiopathic pulmonary fibrosis (IPF) has an incidence of around 7.44 cases per 100,000 inhabitants, while in the United States some series show an incidence of 16.3 cases per 100,000 inhabitants or even 93.7 cases per 100,000 people as described in a systematic review conducted by Hutchinson [4] covering the decade from 2001 to 2011
Results from a study of 186 patients from a single centre in the United States showed that the percentage of patients who had to discontinue treatment with pirfenidone or nintedanib due to adverse events (AE) was similar to that observed in clinical trials (20.9% and 26.3%, respectively), with gastrointestinal AEs being primarily responsible for discontinuation [49]
Summary
Interstitial lung diseases (ILD) are a heterogeneous group of pulmonary disorders characterized by varying degrees of inflammation and fibrosis resulting in the loss of alveolar function and impairment of gas exchange [1]. Patients with IPF present with dyspnea, cough and asthenia, which are symptoms that cause a reduction in daily physical activity and muscle strength leading to a precarious quality of life and often result in social isolation with increased levels of dependence and immobility as the disease progresses and causing a significant number of cardiopulmonary complications. These patients experience depressive and anxiety disorders, creating a situation that is difficult to manage for both patients and their caregivers [11]. We review the main pharmacological interactions of the two currently available antifibrotic drugs, used individually or in combination, as well as some practical aspects of their therapeutic management, which have become more complex in this pandemic
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