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Orphan drug designation granted to nintedanib for treatment of systemic sclerosis

Boehringer Ingelheim has announced that the European Commission (EC) and US Food and Drug Administration (FDA) have granted Orphan Drug Designation to nintedanib for the treatment of systemic sclerosis (SSc, also known as scleroderma), including the associated interstitial lung disease (SSc-ILD).

SENSCIS™ is specifically evaluating nintedanib to understand the disease process and potential benefit of the compound to treat SSc-ILD.1 Systemic sclerosis, commonly referred to as ‘scleroderma,’ is a disfiguring, disabling and potentially fatal rare disease that can cause scarring of the skin, lungs (SSc-ILD) and other organs.2,3 Worldwide an estimated 1 in 3000 people have systemic sclerosis3 (scleroderma) and up to 90% may develop some degree of lung scarring.2 SSc-ILD accounts for 35% of all disease-related deaths.4

Scleroderma and associated interstitial lung disease have a devastating impact on the patient community, and we welcome this important news that a potential new treatment has received Orphan Drug Designation. It’s a crucial step forward in helping to address an unmet need and represents important progress for patients with this rare disease.” said Joep Welling, Federation of European Scleroderma Associations.

“The Orphan Drug Designation granted by both the EC and FDA for nintedanib is an important milestone in the development of much needed therapies for the various complications of scleroderma” said Dr William Mezzanotte, Therapeutic Area Head, Respiratory Medicine at Boehringer Ingelheim.

The need for treatment options for people with SSc-ILD is significant, and the SENSCISTM trial is an important first step in exploring the benefit of nintedanib in addressing the complications of scleroderma. The success of nintedanib in treating IPF, in both clinical trials and the clinical world, along with similarities of IPF to other fibrotic lung diseases, including SSc-ILD, gives us great hope that this important medicine can help patients with SSc-ILD. At Boehringer Ingelheim, we are committed to transforming fibrotic lung diseases from fatal diseases to chronic, treatable ones.”


  1. Boehringer Ingelheim. A Trial to Compare Nintedanib with Placebo for Patients with Scleroderma Related Lung Fibrosis. In: [Internet]. Bethesda (MD): National Library of Medicine (US). 2000 [cited 2015 December 7]. Available from: Last accessed August 2016.
  2. Solomon JJ et al. European Respiratory Update: Scleroderma lung disease. Eur Respir Rev 2013;22:127, 6–19.
  3. University of Michigan Scleroderma Program. What is Scleroderma? Available at: Last accessed August 2016.
  4. Silver, et al. Management of Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD). Rheum Dis Clin North Am. 2015; 41(3):439–57.