S5. E6. 每一口呼吸，都珍貴 BECAUSE EVERY BREATH COUNTS

肺纖維化的疾病成因非常多種；呼吸困難是患者最切身相關的病徵。若無法進行有效治療，病人會因此喪命。百靈佳殷格翰藉由藥品 Nintedanib，取得治療進展的重大突破。

每天早上睜眼的瞬間，我們通常會先深呼吸，舒緩心情、迎接美好的一天。

但是，對特發性肺纖維化（IPF）患者而言，情節就不是這樣了！每一口呼吸都會伴隨不愉快與壓抑的感覺，彷彿穿了一件過緊的外套；對於患有硬皮病（scleroderma）的人而言感受度更差，因為他們身體的皮膚已經呈收縮狀態。

百靈佳殷格翰資深臨床研究人員 Wiebke Sauter 博士說：「被壓縮」這個形容詞，是由全身性硬化症（也稱硬皮病，是一種自體免疫全身性疾病）患者親口說出的真實感受。」Sauter 博士補充道：「這種疾病會導致瘢痕組織（scar tissue）硬化；皮膚與身體器官的硬化令人非常不舒服。」但，這還不是最糟的部分，全身性硬化症有關之間質性肺病（SSc-ILD）也可能隨之發生，導致肺部愈來愈僵硬，瘢痕組織硬化，造成肺纖維化，患者因此呼吸困難，氧氣輸送至血液的功能不停下降。因此，治療這個疾病時，肺部是最需要被重視的器官。

肺部纖維化成因

約有200種潛在疾病會導致肺部纖維化（一種普遍但是致命的綜合病症），除了全身性硬化、類風濕性關節炎等，不同的過敏原與環境毒素，也是可能致病的原因。另外，對於特發性肺纖維化（IPF），醫生更是無法找到確切的原因。雖然原因不相同，但這些病症的結果卻是相同的：硬化的組織會干擾氧氣流入血液，若不加以治療，確診後存活期不超過五年。

呼吸系統疾病研究重點

百靈佳殷格翰已經在呼吸道系統領域進行十多年的研究探索。2014年，百靈佳殷格翰將 Nintedanib 成功上市，為特發性肺纖維化（IPF）患者帶來新的希望，讓他們可以過自己想要的生活。Nintedanib 可以緩解纖維母細胞（fibroblasts，構成瘢痕組織的一種細胞）的生長速度。臨床試驗證明，Nintedanib可以緩減IPF相關的肺部暗疾。百靈佳殷格翰炎症治療方案研發部門負責人 Peter Fang 表示：「Nintedanib 比其他 IPF 療法更有效，能確實改變患者的生活。這項成就，也讓百靈佳殷格翰被視為肺纖維化領域的造浪者。」Fang將 Nintedanib 稱為 『百靈佳殷格翰最偉大的成就之一』。Nintedanib之所以能成功問市，也是基於百靈佳殷格翰在呼吸系統疾病領域，累積已經長達 100 年的專業知識，並擁有一支龐大的跨領域團隊，全力進行呼吸道領域相關的研究。/span>

SSc-ILD和PF-ILD患者的重大突破

Nintedanib 自2014年起就是 IPF 患者的處方用藥。迄今為止，還沒有出現全身性硬化症有關之間質性肺病（SSc-ILD）和漸進性纖維化間質性肺部疾病（ PF-ILD）患者的專用醫療方案。作為第一種也是目前唯一的治療方案， OFEV^® 抑肺纖已在全球50多個國家／地區取得使用許可，得以用於 SSc-ILD 治療，在 40 多個國家准許用於治療 PF-ILD。這是治療多種罕見肺纖維化疾病的轉捩點，統計數據顯示，至 2020 年底，有至少15萬人罹患這類罕見肺部疾病，並接受使用Nintedanib 的治療方案。但，我們不能止步於此。藉由 InPedILDTM 研究計畫，百靈佳殷格翰也在研究 Nintedanib 用於兒童和青少年患者時的劑量，以及安全性。

Peter Fang 是百靈佳殷格翰炎症治療方案研發部門負責人，Wiebke Sauter 博士則負責督導使用 nintedanib 的治療方案與臨床研究。

BECAUSE EVERY BREATH COUNTS

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 blood- stream. If left untreated, life expectancy following diagnosis is no more than five years.

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 InPedILDTM study, Boehringer Ingelheim is now also investigating the dosing and safety profile of nintedanib in children and adolescents.

Peter Fang leads the Thera- peutic Area Inflammation at Boehringer Ingelheim, Dr. Wiebke Sauter oversees a clinical study on treatment with nintedanib.