Boehringer’s Nintedanib Approved in EU for Rare Lung Disease in Adults

Rare Daily Staff

The European Commission approved Boehringer Ingelheim’s nintedanib for the treatment of systemic sclerosis-associated interstitial lung disease in adults.

Systemic sclerosis (SSc), also known as scleroderma, is a disfiguring, disabling, and potentially fatal rare autoimmune disease, which causes scarring of various organs, including the lungs, heart, digestive tract, and kidneys and can lead to life-threatening complications. When the lungs are affected, it can cause interstitial lung disease (ILD), known as SSc-ILD. The onset of SSc typically occurs at a young age, between 25 and 55 years, and ILD can develop early in patients with SSc. ILD is a leading cause of mortality, accounting for almost 35 percent of SSc-related deaths.

“Once fibrosis of the lungs occurs it cannot be reversed. Nintedanib, being the first and only approved treatment for SSc-ILD, is serving a high unmet need making a real positive difference to those living with this life-changing condition,” said Peter Fang, senior vice president and head of therapeutic area inflammation at Boehringer Ingelheim.

Nintedanib is a tyrosine kinase inhibitor targeting key receptors involved in signaling pathways that lead to pulmonary fibrosis. It is already approved in more than 75 countries for the treatment of patients living with idiopathic pulmonary fibrosis–a chronic and ultimately fatal disease characterized by a decline in lung function.

The European Commission’s approval is based on the results of the SENSCIS trial, a phase 3, double-blind, placebo-controlled trial conducted to investigate the efficacy and safety of nintedanib in patients with SSc-ILD. The primary endpoint was the annual rate of decline in forced vital capacity assessed over a 52-week period. Results showed nintedanib slowed the loss of pulmonary function by 44 percent relative to placebo, as measured in forced vital capacity over 52 weeks. The results also showed that nintedanib had a safety and tolerability profile similar to that observed in patients with idiopathic pulmonary fibrosis.

The U.S. Food and Drug Administration approved nintedanib in September 2019 as the first and only therapy to slow the rate of decline in pulmonary function in patients with systemic sclerosis-associated ILD, and so far, regulatory approvals have been granted in Canada, Japan, Brazil, and other countries. Nintedanib is also approved in more than 75 countries for the treatment of idiopathic pulmonary fibrosis.

Photo: Peter Fang, senior vice president and head of therapeutic area inflammation at Boehringer Ingelheim