Advances with pharmacotherapy for the treatment of interstitial lung disease

ABSTRACT

Introduction

In recent decades, the primary focus of pharmaceutical research in interstitial lung diseases (ILD) has been on idiopathic pulmonary fibrosis (IPF). Recently, pharmaceutical development has also focused on other forms of ILDs, including connective tissue diseases associated ILD, fibrotic hypersensitivity pneumonitis, and sarcoidosis.

Areas Covered

The authors summarize the advances in pharmacotherapy for the treatment of ILD. Specifically, the authors review the most recent studies and discuss the most recent research findings and future prospects.

Expert opinion

Data collected over the past years have confirmed the efficacy of antifibrotic drugs on slowing disease progression in IPF. The usual strategy for CTD-ILD management is represented by the combined use of corticosteroids and immunosuppressive agents. There is an urgent need for new target therapies. The concept of progressive fibrosing ILD has emerged in the ILD community in recent years, which has led to grouping several diseases with a common disease behavior to find an effective treatment . At present, selecting the best therapy in ILDs should be reasonably performed on a case-by-case basis through a multidisciplinary team discussion in tertiary ILD centers, taking into consideration patients’ symptoms, lung functional trends, and radiological changes.

KEYWORDS:

• Interstitial lung disease
• idiopathic pulmonary fibrosis
• connective tissue disease associated ILD
• fibrotic hypersensitivity pneumonitis
• sarcoidosis
• progressive fibrosing ILD
• pirfenidone
• nintedanib

Article Highlights

• Nintedanib and pirfenidone have confirmed their safety profile and efficacy in slowing disease progression in idiopathic pulmonary fibrosis.

• Promising novel molecules targeting different pathways are under investigation in IPF.

• Multiple trials are ongoing, testing the combination of immunosuppressive and antifibrotic agents in ILDs in which the activation of proinflammatory signals in the lung parenchyma develops toward the fibro-proliferative pathway.

• Researchers are conceiving different ILDs (fibrotic hypersensitivity pneumonitis, autoimmune ILDs, idiopathic non-specific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, sarcoidosis), with similar clinical features and disease course, as a single entity focusing on antifibrotic therapy even in non-IPF-ILDs.

• Multidisciplinary team discussion (through discussion of patients’ symptoms, lung functional trends, and radiological changes) still represents a valid tool for assessing the best management and therapeutic strategy for patients with ILDs.

Declaration of Interest

G Sgalla has received personal fees from Boehringer Ingelheim outside the submitted work while LRicheldi has received grants and personal fees from Boehringer Ingelheim, as well as personal fees from Roche, Biogen Idec, Sanofi, FibroGen, Promedior, Celgene, RespiVant, Cipla, Zambon, CSL Behring, Nitto, and Pilant Therapeutics outside of the submitted work. … The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer Disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This manuscript has not been funded.