BURLINGTON, ON, Nov. 27, 2019 /CNW/ - Boehringer Ingelheim (Canada) Ltd. announced today that Health Canada has approved OFEV® (nintedanib) as the first and only treatment indicated to slow the rate of decline in pulmonary function in patients with systemic sclerosis associated interstitial lung disease (SSc-ILD).2 Interstitial lung disease (ILD) is the leading cause of death related to systemic sclerosis (SSc) or scleroderma, accounting for 35 per cent of all disease-related deaths.3 This new approval changes the treatment paradigm for SSc-ILD, giving patients an opportunity to slow the progression of decline in lung function where no Health Canada approved options previously existed. "Medical advancements, like the approval of OFEV®, are a rare thing when it comes to treating conditions like systemic sclerosis," said Dr. Martin Kolb, Respirologist at St. Joseph's Healthcare Hamilton and Director, Division of Respirology at McMaster University. "We now have the unprecedented opportunity to care for our patients in a different way that offers hope by slowing down the progression of this disease." Systemic sclerosis is a rare disease estimated to affect up to 40,000 Canadians.4 Interstitial lung disease (ILD) is the leading cause of death among people with systemic sclerosis (SSc) or scleroderma.5 Because scleroderma affects the connective tissue, symptoms can occur in any area of the body including the skin, muscles, blood vessels and internal organs, making it difficult to diagnose.6, 7, 8 More than 80 per cent of patients are women between the ages of 30 to 50.9 "When systemic sclerosis involves the lungs, it can be incredibly debilitating. For years I've watched my patients' disease progress and have been frustrated by the fact that there weren't any approved treatments to slow the disease from progressing," said Dr. Sindhu Johnson, Director of the Toronto Scleroderma Program and Associate Professor of Medicine at the University of Toronto. "I am excited to now be able to offer patients OFEV, which, by slowing the deterioration (or worsening) of the lungs, can help patients to continue to live well despite their disease." OFEV® is currently approved in Canada and more than 70 countries for the treatment of patients living with idiopathic pulmonary fibrosis (IPF), and has been shown to slow IPF progression by reducing the annual rate of decline in lung function, as measured by forced vital capacity (FVC).10 Both IPF and SSc-ILD are conditions known as pulmonary fibrosis, which is when the lungs become scarred and stiff.11 "With OFEV® being the first therapy available in Canada to slow the rate of decline in pulmonary function for patients with SSc-ILD, it provides hope for these patients and their loved ones," said Dr. Uli Brödl, Vice President, Medical and Regulatory Affairs, Boehringer Ingelheim (Canada) Ltd. "This approval is supported by positive evidence from the Phase III SENSCIS study that showed that OFEV significantly slowed the progression of lung disease in this patient population." The SENSCIS® Trial

The approval was based on results of SENSCIS®, a Phase III double-blind randomized, placebo-controlled trial, that involved 576 patients across 32 countries, including Canada. The primary endpoint was the annual rate of decline in FVC in patients with SSc-ILD.12 Results show that OFEV® slowed the loss of pulmonary function by 44% (41 mL/year) in patients with SSc-ILD relative to placebo, as measured by FVC over 52 weeks.13 Results also showed the safety and tolerability of OFEV® in SSc-ILD patients. The most common adverse events (occurring in greater than or equal to 3%) in OFEV®-treated patients compared to placebo included: diarrhea, nausea, vomiting, abdominal pain, liver enzyme elevation, weight decreased, fatigue, decreased appetite, pneumonia, musculoskeletal pain, dizziness and hypertension.14 Both the FDA and Health Canada have granted approval for the application of nintedanib in SSc-ILD. This approval is a part of the company's ongoing commitment to improving the lives of people living with SSc-ILD. Boehringer Ingelheim (Canada) Ltd.

Improving the health of humans and animals is the goal of Boehringer Ingelheim, a research-driven pharmaceutical company. In doing so, the focus 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 top 20 companies in the pharmaceutical industry. Some 50,000 employees create value through innovation daily for the three business areas of 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. The Canadian headquarters of Boehringer Ingelheim was established in 1972 in Montreal, Quebec and is now located in Burlington, Ontario. Boehringer Ingelheim employs approximately 600 people across Canada. More information about Boehringer Ingelheim can be found at www.boehringer-ingelheim.ca or in our annual report: http://annualreport.boehringer-ingelheim.com/. For more information, please contact: Sara McClelland

Boehringer Ingelheim (Canada) Ltd.

sara.mcclelland@boehringer-ingelheim.com

(905) 631-4713 Robbyn Walsh

Proof Inc.

rwalsh@getproof.com

(416) 969-2759 References _________________

1 Ofev® Product Monograph. Boehringer Ingelheim (Canada) Ltd. November 21, 2019.

2 Ofev® Product Monograph. Boehringer Ingelheim (Canada) Ltd. November 21, 2019.

3 Silver KC, Silver RM. Management of Systemic Sclerosis-Associated Interstitial Lung Disease. Rheum Dis Clin North Am. 2015 Aug; 41(3): 439–457. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4515778/

4 Scleroderma Society of Canada. Scleroderma Overview. Available at https://fhs.mcmaster.ca/purr/documents/Scleroderma-Overview.pdf

5 Tyndall AJ et al. "Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database." Ann Rheum Dis. 2010 Oct;69(10):1809-15. doi: 10.1136/ard.2009.114264.

6 Denton, CP, Khanna D. Systemic Sclerosis. Lancet 2017; 390: 1685-99.

7 Cottin V, et al. Interstitial lung disease associated with systemic sclerosis (SSc-ILD). Respir. Res. 2019;20(1):13.

8 US National Library of Medicine. Systemic Scleroderma. Available at: https://ghr.nlm.nih.gov/condition/systemic-scleroderma#genes

9 Scleroderma Society of Canada. Scleroderma Overview. Available at https://fhs.mcmaster.ca/purr/documents/Scleroderma-Overview.pdf

10 Ofev® Product Monograph. Boehringer Ingelheim (Canada) Ltd. November 21, 2019.

11 Canadian Pulmonary Fibrosis Foundation. Understanding PF – What is Pulmonary Fibrosis? Available at https://cpff.ca/understanding-pf/what-is-pulmonary-fibrosis/

12 Distler O, et al. Nintedanib for Systemic Sclerosis – Associated Interstitial Lung Disease. New England Journal of Medicine. 2019; 1-11.

13 Distler O, et al. Nintedanib for Systemic Sclerosis – Associated Interstitial Lung Disease. New England Journal of Medicine. 2019; 1-11.

14 Ofev® Product Monograph. Boehringer Ingelheim (Canada) Ltd. November 21, 2019. SOURCE Boehringer Ingelheim (Canada) Ltd.