Key facts

• IPF causes severe breathlessness in about 15,000 people in the UK, with a life expectancy of only two and a half to three and a half years after diagnosis and no known cause or cure

• Previously only one drug, with variable response, was licensed for treating IPF, Pirfenidone, which slows the progression of the disease

• Nintedanib, a chemotherapeutic was shown to provide an alternative safe and effective treatment for IPF patients unresponsive to Perfenidone

Scarred and stiff lungs

IPF sees inflammation of the air sacs, or alveoli, of the lungs developing into permanent scarring. This scar tissue spreads over time, reducing lung capacity, making it harder to breathe and get enough oxygen into the bloodstream.

Those with the disease become breathless after the most simple of tasks, such as walking,  may develop a constant cough, with the disease progressing to complete lung failure within a few years of diagnosis.

Professor Luca Richeldi’s group found that Nintedanib, originally developed to treat cancer, can slow the progression of IPF by about half, compared to placebo.

Based on their and others’ results, the US Food and Drug Administration licensed Nintedanib for use in IPF treatment in autumn 2014, with the European Union and UK following suit soon after. Their discovery provides new hope for patients who currently have very limited treatment options.

Improving diagnosis

Because current IPF therapies can only slow disease progression, it is important to diagnose as early as possible to give patients greater quality of life for longer. However, early diagnosis remains one of the biggest challenges, with relatively mild and non-specific symptoms.

Because of this Professor Luca Richeldi is collaborating with the Institute of Sound and Vibration Research at University of Southampton to develop an ‘acoustic imaging’ method, where a digital stethoscope is used to take sound recordings from the lungs to diagnose early stage IPF. IPF is also known as ‘Velcro lung’, due to a distinctive sound signature when a stethoscope is used – each breath sounding much like Velcro fasteners being pulled apart – the group is working to validate and automate identification of this sound.

Discovering the cause

Current treatments for IPF, including Nintedanib, only treat the symptoms and not the cause. While it‘s known there is an increased incidence of the disease among smokers and elderly people, the cause of the disease remains unclear.

Professor Luca Richeldi’s group are researching the root causes, drawing on information collected during Nintedanib development, from their acoustic imaging programme, from advanced imaging techniques (such as microCT, in collaboration with the µVIS X-ray Imaging Centre at University of Southampton) and from research patients at their specialist clini