Boehringer files for new rare disease use for nintedanib in EU, US

Boehringer Ingelheim has filed for a new use for its lung drug nintedanib in the US and Europe, bidding to add the rare disease systemic sclerosis to the existing indication for idiopathic pulmonary fibrosis (IPF).

The German pharma said it had filed dossiers with the FDA and the European Medicines Agency (EMA) for nintedanib, for interstitial lung disease associated with systemic sclerosis (SSc-ILD).

Nintedanib is an anti-fibrotic medicine approved in more than 70 countries for treatment of adults with IPF.

Systemic sclerosis, also known as scleroderma, is a rare, immune-mediated rheumatic disease characterised by thickening and scarring (fibrosis) of connective tissue throughout the body, which can be debilitating and possibly life-threatening.

The charity Scleroderma & Raynaud’s UK estimates that around 2.5 million people have sceleroderma worldwide.

Around 80% of patients with scleroderma develop interstitial lung disease, with up to 30% developing to become more serious and progressive.

Next to pulmonary hypertension, interstitial lung disease is one of the leading causes of death for people with SSc.

Dr Susanne Stowasser, associate head of respiratory medicine at Boehringer Ingelheim said: “Systemic sclerosis associated interstitial lung disease has a devastating impact on those it affects – often women in the prime of their lives.

“Pulmonary fibrosis is a key driver of mortality in systemic sclerosis, and currently there are no approved treatments for SSc-ILD. Boehringer Ingelheim is pleased that we are able to take a step closer to potentially bringing an approved treatment for addressing the decline in lung function to patients with this rare condition.”

Branded as Ofev in IPF, nintedanib is an important drug for Boehringer Ingelheim, generating sales of around $620 million in 2017, up 35% on the previous year.

This was a sizeable chunk of the company’s total drug sales, which stood at $7.1 billion in 2017.

The privately-owned German pharma is about the last in the industry to publish its full year results, and updated figures will be available when it announces its 2018 sales figures next month.