The walls of the arteries become thick and stiff, which narrows the space for blood to pass through. This increases blood pressure.
The disease leads to exercise intolerance, breathlessness and heart failure.
It can even stop the right side of the heart from working properly.
In the UK, around experts believe 6,000 to 7,000 people have pulmonary hypertension. Symptoms of the condition include shortness of breath, tiredness, feeling faint or dizzy, chest pain, a racing heartbeat, and swelling in the legs and ankles.
The condition can’t be cured, experts have said.
However, now experts believe a substance could make significant headway to treating the condition.
A lipid (fat) with anti-inflammatory properties has been discovered by Cardiff University, with colleagues from Universities of Pittsburgh, Oregon and Michigan.
It is being developed into a new drug for the treatment of diseases that currently have limited therapeutic options.
Detailed study of the lipid by the groups found it could dampen down inflammation in circulating blood cells, making it a good candidate for development into a drug for several inflammatory diseases.
Now under license to the biopharmaceutical company Complexa, the new drug – CXA-10 – will run in clinical trials where it will be tested on patients with FSGS (a rare disease that attacks the kidneys) and pulmonary arterial hypertension.
FSGS is a rare disease that leads to scarring in the kidney, reducing kidney function and causing the majority of sufferers to develop end-stage renal disease.
Once dialysis is required, the average life expectancy is only eight years.
There are currently no approved therapeutic options for FSGS patients, who often endure long courses of high-dose steroids without responding.
CXA-10 is being investigated as a steroid-sparing agent in recently diagnosed patients.
Professor Valerie O’Donnell, Co-Director of Systems Immunity Research Institute at Cardiff University, said: “The discovery that this lipid has potent anti-inflammatory activity is now being used to develop therapies that could significantly improve the lives of people with life-threatening diseases.”
Josh Tarnoff, president and chief executive officer of Complexa, said: “CXA-10 has already demonstrated disease-modifying effects in preclinical tests and has great potential to do the same in inflammatory conditions such as FSGS and PAH, in which many patients fail to respond to existing treatment options.”
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