Hope for patients with ILD associated scleroderma

The drug nintedanib has been shown to help patients with Interstitial Lung Disease (ILD) associated scleroderma, according to data published in the New England Journal of Medicine.

The trial, led by Professor Toby Maher, a respiratory consultant at Royal Brompton & Harefield NHS Foundation Trust, found that patients with ILD associated scleroderma who were given nintedanib had a slower decline in their lung function compared to the placebo group.

What is Interstitial Lung Disease (ILD) associated scleroderma?

ILD is an umbrella term used for a large group of diseases that cause scarring (fibrosis) of the lungs and includes Idiopathic Pulmonary Fibrosis (IPF) and Hypersensitivity Pneumonitis. 

The scarring in the lungs causes them to become stiff, making it difficult to breathe.

In ILD associated scleroderma the scarring is caused by systemic inflammation in the body. Although certain immunosuppressive therapies can slow down disease progression, there is currently no cure for the disease. 

The results

In the global trial, funded by Boehringer Ingelheim, 576 participants with ILD associated scleroderma were randomly assigned to receive either nintedanib or a placebo, to be taken twice daily. Both the participants and the researchers were “blinded” to who was receiving what treatment. 

Patients with ILD associated scleroderma normally see a gradual decline in their lung function and the results of the study showed that this decline in lung function could be slowed down. 

After 1 year of taking either nintedanib or placebo researchers found the lung function in the treatment group (nintedanib) to have decreased on average by 52.4 ml compared to a 93.3 ml reduction in the non-treatment group (placebo).

What’s next?

The drug nintedanib is already licensed for use in patients with mild to moderate IPF but more data is needed for patients with ILD associated scleroderma. 

This study was double blinded, which means that neither the participants nor the researchers knew which group of patients were receiving the placebo and which group were receiving nintedanib.

The next study, which has already begun, is an open-label trial in which both the participants and researchers know which treatment is being administered. This will help to provide long term data on nintedanib use in this patient group. 

To find out more about this trial or about any of our research studies please contact us.