There are many different causes of pulmonary fibrosis, and breathlessness is the most relevant symptom for patients. Left untreated, it is usually fatal. With its medicine nintedanib, Boehringer Ingelheim has now achieved a major breakthrough.

The moment you open your eyes for the first time each morning is generally followed by a deep breath, a stretch – for a good start into the day. For those who suffer from pulmonary fibrosis, this is different. Every morning, suddenly, there it is again, that unpleasant and oppressive feeling. It is as if you are stuck inside an over-tight jacket. For people who suffer from scleroderma associated lung fibrosis, it is even worse: It also feels as if the skin around your body has contracted. “Those are the words that a patient used to describe their symptoms and to explain what it feels like,” reports Dr. Wiebke Sauter, Senior Clinical Research Scientist at Boehringer Ingelheim. This patient is suffering from systemic sclerosis, also known as scleroderma, a systemic autoimmune disease. “This disease causes connective tissue to harden,” says Sauter. “The skin and other organs become hard and unpleasant.” But that is not the worst aspect of the disease: In conjunction with systemic sclerosis, a type of pulmonary fibrosis known as systemic sclerosis associated interstitial lung disease (SSc-ILD) can occur. “The lung then becomes increasingly stiff and people suffer from breathing problems due to the scar tissue, and its ability to transport oxygen into the bloodstream continuously declines. SSc-ILD is the most frequent cause of death among scleroderma sufferers,” the scientist explains. “That is why the lung is the most important organ in the treatment of the disease.”

Many Different Causes of Pulmonary Fibrosis

Around 200 underlying diseases can cause this rare and generally fatal syndrome. Besides systemic sclerosis, these also include rheumatoid arthritis. However, allergens and environmental toxins are also possible causes. In the case of idiopathic pulmonary fibrosis (IPF), doctors are not able to identify any cause at all. The consequences are always the same, however: Hardened tissue makes it increasingly difficult for the lung to transport oxygen into the bloodstream. If left untreated, life expectancy following diagnosis is no more than five years.

Peter Fang leads the Therapeutic Area Inflammation at Boehringer Ingelheim, Dr. Wiebke Sauter oversees a clinical study on treatment with nintedanib.
Research Focus on Respiratory Diseases

Boehringer Ingelheim has been conducting research in this field for more than ten years. In 2014, the pharmaceutical company introduced the substance nintedanib to the market and provided people with an IPF diagnosis with fresh hope to be able to live the life they want. Nintedanib can slow down the growth of fibroblasts, the cell type that the characteristic scar tissue consists of. Clinical trials have shown that the medicine can slow down the insidious loss of lung function associated with IPF. “More than any other IPF therapy, this product influences patients’ lives,” says Peter Fang, Head of Therapeutic Area Inflammation at Boehringer Ingelheim. “It has also contributed to our recognition as a leader in the field of pulmonary fibrosis.” He calls nintedanib “one of Boehringer Ingelheim’s greatest successes.” It was only possible because the company has now 100 years of expertise in the area of respiratory diseases and has pioneered research on this problem with a large, interdisciplinary team.

Major Breakthrough for SSc-ILD and PF-ILD Patients

Nintedanib has been helping patients with an IPF diagnosis since 2014. No approved treatment option has existed to date for people who suffer from SSc-ILD and other chronic fibrosing interstitial lung diseases with a progressive phenotype (PF-ILDs). As the first and only therapy, OFEV® is now approved in more than 50 countries for the treatment of SSc-ILD and in more than 40 countries for the treatment of PF-ILDs. This marks a turning point in the treatment of a wide range of rare forms of pulmonary fibrosis, and it is estimated that over 150,000 people with these rare lung conditions will have been treated with nintedanib worldwide by the end of 2020. But there is still further therapeutic need. With the InPedILD™ study, Boehringer Ingelheim is now also investigating the dosing and safety profile of nintedanib in children and adolescents.


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