Interstitial lung disease

Adverse effect

Nintedanib

Idiopathic pulmonary fibrosis

Internal medicine

Tolerability

Medicine

Lung

Pirfenidone

Pathology

Drugs

Progressive fibrosing interstitial lung diseases (ILDs) involve similar pathophysiological processes, indicating the potential for common approaches to treatment. Nintedanib (Ofev®), an intracellular tyrosine kinase inhibitor (TKI) with antifibrotic properties, was one of the first drugs approved for use in idiopathic pulmonary fibrosis (IPF) and has more recently been approved for use in other chronic fibrosing ILDs with a progressive phenotype and systemic sclerosis-associated ILD (SSc-ILD). In multinational phase III trials, nintedanib significantly reduced the annual rate of decline in forced vital capacity (FVC) in adults with IPF, other progressive fibrosing ILDs and SSc-ILD. Reductions in FVC decline with nintedanib in patients with IPF and severe gas exchange impairment were comparable to those in patients with milder disease. Real-world experience in patients with IPF supports the effectiveness of nintedanib in slowing ILD progression. Nintedanib had a manageable tolerability profile in patients with fibrotic ILDs in clinical trials and real-world studies. No new safety signals have emerged from global pharmacovigilance data. Nintedanib continues to represent an important therapeutic option in patients with IPF and is the first drug to be approved for use in patients with other chronic fibrosing ILDs with a progressive phenotype or SSc-ILD, with these approvals expanding the range of fibrotic ILDs for which nintedanib can be prescribed.Treatment options for fibrotic interstitial lung diseases (ILDs) that involve progressive lung function decline have historically been limited. Nintedanib (Ofev®) was one of the first antifibrotic drugs to be approved for use in idiopathic pulmonary fibrosis and is now also approved for use in other chronic fibrosing ILDs with a progressive phenotype and systemic sclerosis-associated ILD (SSc-ILD). Nintedanib reduced lung function deterioration in patients with idiopathic pulmonary fibrosis, other chronic fibrosing ILDs with a progressive phenotype and SSc-ILD in well-designed clinical trials. In patients with idiopathic pulmonary fibrosis, the clinical benefit of nintedanib was shown to persist over more than 4 years of treatment. The most common adverse events in nintedanib recipients were diarrhoea and nausea, which were manageable in the majority of patients. Real-world experience supports the effectiveness and acceptable safety of nintedanib. Nintedanib remains an important treatment option for patients with idiopathic pulmonary fibrosis and is the first drug to be approved for use in patients with other chronic fibrosing ILDs with a progressive phenotype and SSc-ILD.

Nintedanib: A Review in Fibrotic Interstitial Lung Diseases