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Idiopathic pulmonary fibrosis (IPF) is a form of interstitial lung disease, which means that it affects the space around the alveoli inside the lungs. IPF is characterised by progressive loss of lung function, dyspnoea and cough, and substantial impairments in quality of life.1 2 IPF is the most common form of interstitial lung disease but is still a rare disease. A study of healthcare claims data in the USA from 2004 to 2010 suggested an incidence of 19.3 cases per 100 000 person-years in people aged 55–64 years.3 IPF is more common in older people, in men and in former smokers.1

The clinical course of IPF is variable, but its prognosis is poor, with historical data suggesting a median survival time after diagnosis of 2–3 years.1 Acute, clinically significant, respiratory deteriorations characterised by evidence of new, widespread alveolar abnormality are known as acute exacerbations of IPF.4 Such acute deteriorations in respiratory function, which are unpredictable and often of unknown cause, are a major cause of morbidity and mortality in patients with IPF.5

Why is this medication prescribed?

Nintedanib is used for the treatment of idiopathic pulmonary fibrosis (scarring of the lungs with an unknown cause). Nintedanib is in a class of medications called kinase inhibitors. It works by blocking the action of enzymes involved in causing fibrosis.

How should this medicine be used?

Nintedanib comes as a capsule to take by mouth. It is usually taken with food every 12 hours (twice a day). Take nintedanib capsules at around the same times every day. Follow the directions on your prescription label carefully, and ask your doctor or pharmacist to explain any part you do not understand. Take nintedanib exactly as directed. Do not take more or less of it or take it more often than prescribed by your doctor.

How Ofev (nintedanib) works

Ofev inhibits tyrosine kinase, an enzyme essential for signal transduction pathways that trigger growth factors like vascular endothelial growth factor receptor (VEGFR), fibroblast growth factor receptor (FGFR), and platelet derived growth factor receptor (PDGFR), which are secreted in excess amounts in patients with IPF.

These growth factors promote collagen production and deposition and control the differentiation, maturation, and proliferation of the fibroblasts, which eventually lead to the formation of scar tissue in the lungs.

Ofev binds to the phosphorylating site of tyrosine kinase, which blocks the cell signaling mechanism, slowing the progression of the disease.

Clinical Evaluation

At the time of diagnosis, a full medical history was obtained, including previous and concomitant medication use and details of comorbidities. Prior to the initiation of nintedanib, pulmonary function tests were performed assessing FVC, forced expiratory volume in 1 s, single-breath diffusing capacity of the lung for carbon monoxide (DLCO), 6-min walk test, and high-resolution computed tomography [20]. The details of all previous treatments for IPF were recorded, along with reasons for switching to nintedanib if applicable.


 Nintedanib seems to be an effective treatment option with tolerable side effects in the real life setting, however longer observation is required.