An overview of the disease
Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scar tissue growing inside a patient’s lungs and making it hard to breathe. It is a progressive disease that gets worse over time as the lungs cannot take in enough oxygen, causing shortness of breath and resulting in life-threatening complications such as respiratory failure.
The actual cause of the disease is not fully understood. However, common risk factors include family history (genetics), cigarette smoking, gastroesophageal reflux disease (acid reflux), gender (being male) and age amongst others.
Symptoms
IPF mainly includes two symptoms:
- Breathlessness: also called dyspnoea. It occurs when a person feels difficulty in breathing. Breathlessness or dyspnoea can affect day-to-day activities such as walking, exercising, going up the stairs, and getting dressed amongst other activities.
- Chronic cough: a cough that has remained for longer than 8 weeks. This is usually a dry cough (cough does not produce mucus) but some people may also cough up sputum.
Other symptoms include:
- Chest pain
- Unintentional weight loss
- Anxiety
- Rounded shape fingers called clubbing
- Tiredness
- Loss of appetite
- Loss of energy
Treatment
There is currently no therapy available to fully cure IPF. The main aim is to slow down disease progression and to minimise the symptoms as much as possible. As a disease becomes more advanced palliative care will be offered to the patients.
- Self-care – Stop smoking, daily exercise, eat a healthy and balanced diet, try to keep a distance from people with a chest infection and a cold.
- Medicines – currently there are only two medicines used to slow down disease progression. These are:
- Esbriet (Pirfenidone)
- Ofev (Nintedanib)
IPF market outlook
According to 2020 forecast publications (link 1, link 2), the IPF market will reach $3.6bn by 2029 with a compound annual growth rate of 3.8% across the seven major markets (forecast period 2020 -2029). The main growth drivers are the increasing prevalence of fibrosis disease and the rising geriatric population across the globe. Moreover, changing lifestyles and rising consumption of nicotine such as cigarettes are providing a boost to this market growth.
Currently, the IPF market holds two approved drugs; Esbriet (Pirfenidone) and Ofev (Nintedanib) both of which aim at slowing down disease progression by acting as anti-fibrotic mediators. Nintedanib is developed by Boehringer Ingelheim (approved by the US FDA in 2014) – a powerful intracellular tyrosine kinase receptor inhibitor of platelet-derived growth factor receptors (PDGF-R), Fibroblast growth factor receptors (FGFRs), vascular endothelial growth factor (VEGF), and non-receptor tyrosine kinases of the Src family drug.
Pirfenidone was developed by Intermune, which is now part of Roche pharmaceuticals. The drug is responsible for the inhibition of fibroblast proliferation and attenuated transforming growth factor-beta (TGF-beta)-induced alpha-smooth muscle actin expression at both protein and mRNA levels. Furthermore, pirfenidone reduces TGF-β-induced phosphorylation of Akt, Smad3, and p38 which are the key mediators of the TGF-β pathway.
Idiopathic pulmonary fibrosis drug uptake
The global IPF market is expected to grow significantly due to emerging treatments and techniques. Moreover, rising demand for IPF therapy globally and meaningful numbers of advancements in medical science would result in nurturing the growth of the market. Also, the increasing prevalence of IPF due to the surge in the cigarette smoking population boosts the market growth.
The major challenge in the IPF disease space comes with the drug development process. In pre-clinical trials, several promising candidates were based on animal model data but did not show efficacy in human clinical trials. Moreover, other immunological treatments have not shown efficacy in IPF patients.
The major key drivers for the growth in IPF space will be the launches of the following five new pipeline agents:
|
Company |
Drug | Status |
|
Fibrogen |
pamrevlumab |
Phase III |
|
Hoffmann-La Roche |
PRM-151 |
Phase II |
|
Galecto Biotech AB |
TD-139/GB-0139 |
Phase IIb |
|
Celgene |
CC-90001 |
Phase II |
| Liminal Bioscience |
PBI-4050 |
Phase II |
Key takeaways
The space of IPF is expected to grow massively to fulfill the high unmet needs with the growing prevalence of IPF disease. The current therapies are only able to slow down disease progression but side effects of the existing therapies always create a need for new drugs in the market. The launch of novel therapies provides ample opportunities to capture a larger market share of IPF disease space. As the cause of IPF is not completely known therefore there is always scope for advancement in research and clinical activity in the IPF space.
#IPF #ILD #raredisease
Sources
https://www.globaldata.com/global-ipf-market-set-reach-3-6bn-2029/
https://rarediseases.org/rare-diseases/idiopathic-pulmonary-fibrosis/
https://www.researchandmarkets.com/reports/4564655/idiopathic-pulmonary-fibrosis-ipf-market