Ingelheim, Germany, 28 February?2020?? Boehringer Ingelheim today announced that the Committee for Medicinal Products for Human Use (CHMP) of the European Medicines Agency (EMA) has adopted a positive opinion recommending granting marketing authorisation for nintedanib for the treatment of systemic sclerosis-associated interstitial lung disease (SSc-ILD) in adults.¹?In September, the U.S. Food and Drug Administration (FDA) approved nintedanib as the first and only medicine to slow the rate of decline in pulmonary function in adults living with SSc-ILD. Regulatory approvals for the treatment of patients living with SSc-ILD have also been granted in other countries including Canada, Japan and Brazil. Systemic sclerosis (SSc), also known as scleroderma, is a disfiguring, disabling and potentially fatal rare autoimmune disease.^3,4,5?It causes scarring (fibrosis) of various organs, including the lungs, heart, digestive tract and kidneys and can have life-threatening complications. When SSc affects the lungs, it can cause interstitial lung disease (ILD), known as SSc-ILD.^2,6? ILD is the leading cause of mortality in SSc, accounting for almost 35% of SSc-related deaths.⁷ ?SSc-ILD is a life-changing disease. Once fibrosis of the lungs occurs it cannot be reversed, and this can have a devastating impact on a patient?s life and daily activities? said Peter Fang, Senior Vice President and Head of Therapeutic Area Inflammation at Boehringer Ingelheim. ?Striving to improve the lives of people living with pulmonary fibrosis, we are pleased with the Committee?s positive opinion, which represents an important step forward in helping to slow down the progression of this rare and life-changing disease.? The positive opinion was based on the results of the SENSCIS^??trial, a phase III, double-blind, placebo-controlled trial conducted to investigate the efficacy and safety of nintedanib in patients with ILD-associated systemic sclerosis (SSc-ILD).²?The primary endpoint was the annual rate of decline in Forced Vital Capacity (FVC) assessed over a 52-week period. Patients remained on the study treatment for at least 52 weeks of treatment or for a maximum of 100 weeks of treatment. Results showed nintedanib slowed the loss of pulmonary function by 44% (41mL/year) relative to placebo, as measured in FVC over 52 weeks.²?The adverse-event profile of nintedanib was similar to that observed in patients with idiopathic pulmonary fibrosis, with the most common adverse event being diarrhoea.² Notes to Editors SENSCIS^??Trial SENSCIS^??was the largest randomised controlled trial to be conducted in patients with SSc-ILD, involving 576 patients across more than 32 countries including the United States, Canada, China, Japan, Germany, France and the United Kingdom. The primary endpoint was the annual rate of decline in lung function as measured in FVC (mL/year) assessed over 52 weeks. The trial also collected data on other manifestations of the disease with key secondary endpoints identified as skin thickness as measured by absolute changes from baseline in modified Rodnan skin score (mRSS) and health-related quality of life measured by the total score on the St George?s Respiratory Questionnaire (SGRQ) at week 52. Enrolment criteria included a diagnosis of SSc with onset of first non-Raynaud symptoms within 7 years, ILD confirmed by high resolution computed tomographic that showed fibrosis affecting at least 10% of the lungs, at least 40% predicted FVC, and a diffusion capacity of the lung for carbon monoxide (DLco) as 30?89% predicted.? Patients were randomised to receive nintedanib 150 mg twice daily or placebo. Patients on stable therapy with mycophenolate or methotrexate and/or taking prednisone up to 10 mg/day were allowed to participate.² About systemic sclerosis-associated ILD Systemic sclerosis-associated ILD is a chronic lung disease in which scar tissue (?fibrosis?) and/or inflammation builds up in the walls of the air sacs of the lungs in a person with a diagnosis of scleroderma.⁸?The disease impacts four times as many women as men, and the onset of the disease typically occurs at a young age, between 25 and 55 years.⁹ Nintedanib? Nintedanib is already approved in more than 70 countries for the treatment of patients living with idiopathic pulmonary fibrosis (IPF) ? a chronic and ultimately fatal disease characterised by a decline in lung function. It is estimated that over 80,000 people with IPF have been treated with nintedanib. Nintedanib is one of two antifibrotic drugs shown to slow the disease progression in IPF, and they are approved and recommended for use in IPF patients by international guidelines.^2? In September 2019, nintedanib was approved in the U.S. as the first and only therapy to slow the rate of decline in pulmonary function in patients with systemic sclerosis-associated ILD. Submissions have been made to other regulatory bodies across the globe and so far, regulatory approvals have been granted in Canada, Japan, Brazil and other countries. Boehringer Ingelheim Improving the health of humans and animals is the goal of the research-driven pharmaceutical company Boehringer Ingelheim. The focus in doing so is on diseases for which no satisfactory treatment option exists to date. The company therefore concentrates on developing innovative therapies that can extend patients? lives. In animal health, Boehringer Ingelheim stands for advanced prevention. Family-owned since it was established in 1885, Boehringer Ingelheim is one of the pharmaceutical industry?s top 20 companies. Some 50,000 employees create value through innovation daily for the three business areas human pharmaceuticals, animal health and biopharmaceuticals. In 2018, Boehringer Ingelheim achieved net sales of around 17.5 billion euros. R&D expenditure of almost 3.2 billion euros, corresponded to 18.1 per cent of net sales. As a family-owned company, Boehringer Ingelheim plans in generations and focuses on long-term success. The company therefore aims at organic growth from its own resources with simultaneous openness to partnerships and strategic alliances in research. In everything it does, Boehringer Ingelheim naturally adopts responsibility towards mankind and the environment. More information about Boehringer Ingelheim can be found on?www.boehringer-ingelheim.com?or in our annual report:?http://annualreport.boehringer-ingelheim.com. Intended audiences This press release is issued from our Corporate Headquarters in Ingelheim, Germany and is intended to provide information about our global business. Please be aware that information relating to the approval status and labels of approved products may vary from country to country, and a country-specific press release on this topic may have been issued in the countries where we do business.