The European Commission and the US Food and Drug Administration have both granted Orphan Drug Designation to Boehringer Ingelheim's Ofev (nintedanib) for the treatment of systemic sclerosis, including the associated interstitial lung disease (SSc-ILD).

Systemic sclerosis, commonly referred to as "scleroderma," is a disfiguring, disabling and potentially fatal rare disease that can cause scarring of the skin, lungs and other organs.

It is estimated that around two million people around the globe have the condition, and up to 90 percent may develop some degree of lung scarring, which is linked with a poor prognosis and accounts for 35 percent of all disease-related deaths.

Orphan Drug status is generally granted by European regulators for a treatment targeting a life-threatening or chronically debilitating disease affecting no more than five people in 10,000, and for which there are no or limited options or the medicine could offer a significant benefit. In the US, it can be awarded to investigational compounds intended for treatment, diagnosis or prevention of rare diseases or disorders affecting less than 200,000 people.

"To have nintedanib receive Orphan Drug Designation is an exciting step forward for people living with scleroderma and associated interstitial lung disease, as well as their families," said Robert Riggs, CEO, Scleroderma Foundation.

"This designation represents important progress towards addressing an unmet need and bringing a potential new treatment to those with this rare and devastating disease," added the group's chairman Luke Evnin.

Ofev is already approved to treat lung-scarring disease idiopathic pulmonary fibrosis, and also for lung cancer under the trade name Vargatef.