Idiopathic Pulmonary Fibrosis - A highly fatal and incurable disease
Idiopathic Pulmonary Fibrosis (IPF) is a diffuse progressive parenchymal lung disease of unknown etiology characterised by fibrotic interstitial infiltrates, with chronic progressive scarring of the gas-exchanging alveoli in the lungs. Although the cause of the disease remains unknown, risk factors include age, family history of IPF, cigarette smoking, medicines, acid reflux, viruses and environment exposures e.g. to feathers, asbestos and silica.
While the course of the disease is variable, the prognosis is uniformly poor, with median survival of about 2-3 years after diagnosis. Most patients with IPF die of respiratory failure (80%), others die of pulmonary hypertension, heart failure, pulmonary embolism, pneumonia and lung cancer.
Patients with IPF typically experience slow progressive worsening of lung function over time due to the chronic progressive scarring, but some experience rapid declines and frequent hospitalisations in the late stages of the disease. IPF is the most common type of interstitial lung disease, estimated to affect between 132,000 and 200,000 people in the US. It is typically seen in older adults, more commonly in men than women, and usually occurs between 50 and 70 years of age.
Our lead product, IPF-Lamellasome, has been engineered to provide a safe treatment option for IPF. IPF-Lamellasome has been optimised to reach the site of alveolar fibrosis. IPF-Lamellasome has demonstrated the ability to significantly decrease well established markers of pulmonary fibrosis in vivo in a large animal model by reducing both myofibroblasts and collagen production1.
IPF-Lamellasome is now ready to move into the clinic.
IPF-Lamellasome has the potential to become first line therapy and the new standard of care
Heatlhcare costs related to IPF are high, and the current Standard of care (SoC) (Esbriet and OFEV) is not curative. It is limited to the relief of symptoms and slowing the progression of the disease by reducing the rate of scarring, with steroids and other immunosuppressants used to try and suppress inflammation. Toxic side effects are also a major issue, with between 21%-26% of patients having to cease treatment, despite the fatal predicted outcome. Furthermore, the current treatments are not approved for early disease, stage at which 50% of patients are diagnosed, and treatment must stop if the disease progresses.
IPF-Lamellasome has therefore a clear potential improve the treatment of IPF by:
These pulmonary antifibrotic properties of IPF-Lamellasome are applicable to a range of other interstitial lung diseases such as Drug-induced Interstitial Lung Disease (ILD), Acute Lung Injury (ALI), Acute Respiratory Distress Syndrome (ARDS) and Radiotherapy-Induced Lung Injury (RILI).