Rare lung diseases

Background

Healthy lungs are a pair of spongy, air-filled organs. Inhaled air travels into the lungs via the trachea and its tubular branches, the bronchi. These divide into smaller branches, the bronchioles, and eventually these bronchioles end in microscopic air sacs, the alveoli. Oxygen from the air is absorbed through the microscopically thin walls of the alveoli into the blood, and carbon dioxide travels from the blood into the alveoli, to be exhaled. The interstitium (the supporting framework of the lungs) is a thin layer of cells between the alveoli, which contains blood vessels and cells that support the alveoli.

Fibrotic lung disease

In patients with fibrotic lung disease, the lung tissue becomes thickened, stiff, and scarred. This results in less efficient absorption of oxygen, and breathing becomes increasingly difficult as the disease progresses, leading to dyspnoea. Severe, persistent dry cough is another particularly troubling symptom and correlates strongly with disease progression in conditions such as idiopathic pulmonary fibrosis (IPF). Other possible symptoms include fatigue, weight loss, aching muscles and joints, and widening and rounding of the fingertips.

https://vicorepharma.com/wp-content/uploads/2019/05/tabs-2-and-6-illustration-fibrotic-lung-disease.jpg Fibrotic lung disease.

Prognosis

Damage caused by fibrosis in the lung cannot be repaired, and patients have very limited therapeutic options. Quality of life is significantly impaired as the disease progresses and the prognosis is poor, with a life expectancy of 3-5 years after diagnosis in patients with IPF. The five-year survival rate for IPF is less than that for many cancers, including bladder, prostate, breast, thyroid, and colon cancer.

Increased pressure in the arteries of the lungs due to vascular compression by scar tissue and other pathological vascular changes can lead to pulmonary hypertension (PH) and subsequently to right heart failure. Pulmonary hypertension is a rare, often fatal, complication of lung diseases such as IPF.

The main immediate cause of death in patients with IPF is the disease itself (over 60% of patients), followed by coronary heart disease (around 15%). Women are less likely to die from the disease itself, and death from pneumonia is almost 10 times more common in men than in women. Another underlying cause of death is lung cancer, which is more likely to be the underlying cause of death in ex- and current smokers than in non-smokers.

The course of fibrotic lung disease.

Because of the debilitating symptoms and poor prognosis, patients face an uncertain future, and patients and their families or caregivers face daily challenges in terms of managing the disease and maintaining an acceptable quality of life.

At present, patients often have a tortuous path to a diagnosis of IPF, with little information about the disease when it is diagnosed, particularly if they are not treated at a specialist centre. Many patients do not immediately realize the gravity of their condition, and both patients and their families or caregivers can later be overwhelmed by the physical and psychological symptom burden, with relentlessly deteriorating quality of life and a continuous increase in distressing symptoms. For approximately 80% of patients, lung transplantation will not be possible because suitable donors are not available, and they currently have few other treatment options. The scientific literature supports the benefits of palliative care, but this is offered infrequently and usually late in the disease process.

Our sole focus at Vicore Pharma is to develop therapies for patients who are affected by fibrotic lung disease, a life-changing condition.

Fibrotic lung disease consists of several different disorders including:

  • Idiopathic pulmonary fibrosis
  • Systemic sclerosis  (lung manifestations)
  • Pulmonary sarcoidosis

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is the most common type of pulmonary fibrosis, and is a severe and devastating disease with no known cause. Debilitating symptoms of dyspnoea and severe, persistent dry cough typically appear between the ages of 50 and 70 years and, while the disease is more common in men, the number of cases in women is increasing.

The prevalence of pulmonary hypertension (PH) in people with IPF is difficult to ascertain, as estimates vary widely depending on the method used to identify PH. Right heart catheterization is the gold standard for the detection of PH.

Incidence and prevalence

The European Idiopathic Pulmonary Fibrosis and Related Disorders Foundation has estimated that between 80,000 and 111,000 people in the EU are currently living with IPF, with up to 35,000 new cases being diagnosed each year. In the USA, approximately 100,000 people are currently living with IPF according to the National Institutes of Health (NIH), with 30,000-40,000 new diagnoses per year. The NIH has estimated the overall prevalence worldwide to be 13-20/100,000 people.

Diagnosis and disease progression

Diagnosis is often delayed because the symptoms of IPF are similar to those of other lung diseases:

  • Lung function testing, including spirometry, provides information on the amount of air that the lungs can hold and how forcefully air can be exhaled, which will indicate the severity of the disease
  • A high-resolution CT scan can confirm the extent of lung scarring

Although IPF is progressive, the rate of scar tissue formation in the lungs varies among patients. The prognosis for patients diagnosed with IPF is usually poor, with patients surviving for only 3-5 years after diagnosis.

Treatment options

Currently, there is no cure for IPF, and treatment options are limited:

  • Two medicines have been approved for use in IPF: pirfenidone and nintedanib. Both have been shown to slow the progression of the disease; however, associated side-effects have limited their use
  • Although the antifibrotic drugs for IPF have a positive effect on disease progression, their effect on symptom control (e.g. cough) or quality of life is limited and not fully explored
  • Supportive treatments include oxygen therapy, and immunization against pneumonia and influenza to avoid infections

Patients are advised to keep as active as possible and to follow a healthy diet with plenty of rest. Clearly, new treatments are urgently needed to reduce the significant burden of this disease.

Vicorepharma

On vicorepharma.com, we use so-called cookies to analyze how our website is used in order to optimize and improve it. You choose yourself whether you want to consent to our use of cookies.

Vicorepharma is responsible for placing cookies on the website and for the processing of your personal data collected by them. For more information about our processing of your personal data, see our privacy policy

Cookie policy

Your consent applies to the following domain: vicorepharma.com
The cookie policy was last updated: 2023-06-29

Privacy Policy

Show the cookies used on our website

Necessary cookies is needed in order for the website's basic functions to work correctly and for us to be able to save your choice of cookies.

  • cookies_set

    Purpose: Saves the visitor’s choice of cookies.
    Issued by: mk cookie master
    Lifespan: 1 year

    Domain: vicorepharma.com

  • selected_cookies

    Purpose: Saves the visitor’s choice of cookies.
    Issued by: mk cookie master
    Lifespan: 1 year

    Domain: vicorepharma.com

Statistical cookies helps the website owners to understand how visitors interact with the website by collecting and report this information.

  • _ga

    Purpose: Used to understand how visitors’ navigate around the website.
    Issued by: Google Analytics
    Lifespan: 2 years

    Domain: vicorepharma.com

  • _gat

    Purpose: Used to throttle request rate. If Google Analytics is deployed via Google Tag Manager, this cookie will be named _dc_gtm_.
    Issued by: Google Analytics
    Lifespan: 1 minute

    Domain: vicorepharma.com

  • _gid

    Purpose: Used to understand how visitors’ navigate around the website.
    Issued by: Google Analytics
    Lifespan: 1 day

    Domain: vicorepharma.com

 

What are cookies?

A cookie is a small text file that is placed on your device (computer/mobile/tablet that has access to the internet) by the website you visit – if you give your consent to this. The cookie can, for example, recognize you as a user when you visit our website and make it possible to remember important information that makes your visit more comfortable and provides an improved user experience. Cookies are so-called passive files that cannot spread dangerous software or viruses on your mobile device or computer.

There are two types of cookies, permanent cookies and session cookies. At vicorepharma.com we use both permanent cookies and session cookies. Permanent cookies are saved as a file on the computer for a certain time until the server that placed them, or you as a visitor choose to delete them. Session cookies are temporarily stored when you as a visitor are on the website and disappear when you close your browser.

Cookies can also be divided into first-party cookies and third-party cookies. First-party cookies are placed by the website you are visiting, while third-party cookies are placed by another website. At vicorepharma.com, we use both first-party cookies and third-party cookies.

 

How to avoid cookies

When you visit our website, you can refuse all or certain types of cookies. You can also withdraw or change your consent at any time by clicking here.

You can also block cookies by changing the settings in your browser. Then your browser will automatically refuse cookies or inform you when a website requests to store cookies.

After your visit, you can review and delete the permanent cookies that have been stored in your browser. In the list below, you will see the name of the cookies vicorepharma.com saves so that you can easily identify them.

Here you will find more information about how to manage cookies in your browser:

 

Cookies used by third parties

On vicorepharma.com there may be content that is embedded from YouTube. YouTube places cookies in your browser when you visit youtube.com or when you click on embedded content from YouTube on our website. vicorepharma cannot control these cookies or the personal data and information that may be collected. vicorepharma does not use the targeted marketing services offered by YouTube. As a user, you therefore need to check YouTube’s website for further information on how they manage cookies, what information they collect and how you can delete any third-party cookies.

 

Cookies used on vicorepharma.com

Necessary cookies is needed in order for the website's basic functions to work correctly and for us to be able to save your choice of cookies.

  • cookies_set

    Purpose: Saves the visitor’s choice of cookies.
    Issued by: mk cookie master
    Lifespan: 1 year

    Domain: vicorepharma.com

  • selected_cookies

    Purpose: Saves the visitor’s choice of cookies.
    Issued by: mk cookie master
    Lifespan: 1 year

    Domain: vicorepharma.com

Statistical cookies helps the website owners to understand how visitors interact with the website by collecting and report this information.

  • _ga

    Purpose: Used to understand how visitors’ navigate around the website.
    Issued by: Google Analytics
    Lifespan: 2 years

    Domain: vicorepharma.com

  • _gat

    Purpose: Used to throttle request rate. If Google Analytics is deployed via Google Tag Manager, this cookie will be named _dc_gtm_.
    Issued by: Google Analytics
    Lifespan: 1 minute

    Domain: vicorepharma.com

  • _gid

    Purpose: Used to understand how visitors’ navigate around the website.
    Issued by: Google Analytics
    Lifespan: 1 day

    Domain: vicorepharma.com

General

Vicore Pharma Holding AB (“Vicore”) shall only process personal data in accordance with applicable data protection law and high industry standards.

 

Personal data.

Vicore processes personal data provided to us;
(i) by you in the day-to-day communications, or

(ii) in connection with entering into or performing an agreement or business arrangement with you, or
(iii) by a third party allowed by law or your consent.

The personal data may consist of contact and identification details and other information relevant to the situation.

 

Purpose.

Vicore process personal data for the purpose of communicating with you and to perform, manage and comply with agreements between us as well as rights and obligations which are allowed by law or applicable regulation.

 

Legal grounds.

The legal grounds for processing personal data is either;
(i) your consent which shall be considered provided when you either voluntary submit the personal data or enter into an agreement with us, or
(ii) a legitimate interest allowed under law.

 

Security

Vicore shall use adequate technical and organisational security measures to protect the personal data from loss and to safeguard against access from unauthorised persons.

Transfers of personal data may only occur;
(i) to third parties who perform services on Vicore behalf and who may only process personal data in accordance with our instructions, and may not use personal data for their own purposes; and
(ii) outside the EU/EEA only in accordance with applicable data protection laws and subject to the EU Commission’s standard contractual clauses, and
(iii) as otherwise permitted by law or your consent.

Duration.

The duration we process personal data is limited to what is reasonable for the purpose of the processing, unless otherwise required or permitted by law.

 

Rights

Vicore is the controller of the personal data processing, meaning that we are responsible for that the personal data is processed correctly and in accordance with applicable data protection laws.

Unless prevented by applicable law, regulation or agreement data subjects have the right to;
(i) know what personal data we process about them,
(ii) request that we rectify or erase inaccurate or incomplete personal data
(iii) object to specific processing of personal data, and
(iv) receive the personal data provided by them and have the personal data transferred to another party responsible for data processing.

All communications with Vicore regarding how we process personal data or exercise of any of your rights can be sent by e-mail to info@vicorepharma.com or by post to the address above.

Reports and complaints can also be directed to Datainspektionen who is the supervisory authority for our processing of personal data.