Actelion has announced that NHS patients in England, who have the rare, incurable and devastating disease pulmonary arterial hypertension (PAH) can now be treated with Uptravi (selexipag).

The drug is an oral treatment that specifically targets the prostacyclin pathway – a major disease pathway involved in the development of PAH.

The decision comes after a Phase III clinical study of more than 1,000 people with the condition, in which it was shown shown to improve long-term outcomes for patients with PAH. A 40% reduction in the risk of a primary outcome event was seen in the patients.

NHS England said it will fund treatment of Uptravi for use in adults with one of the more severe forms of the disease who remain insufficiently controlled despite receiving oral treatment with two other classes of PAH medicines.

The decision means that access is now available for patients in England, catching up to the rest of the UK, where treatment is already available.

“Until today, we have seen an untenable situation where patients in Scotland, Wales and Northern Ireland had access to this medicine, while those in England did not,’ said Dr Iain Armstrong, chair of Pulmonary Hypertension Association UK.

He continued, “We are therefore delighted that patients across the UK can now access selexipag, regardless of postcode. PAH patients suffer from considerable life-limiting symptoms including breathlessness which can prevent normal everyday activities, and having access to new treatments that are shown to be effective is vital.”

PAH affects around 3,000 people in the UK, caused by a narrowing of the blood vessels that lead from the right side of the heart to the lungs, resulting in increased blood pressure in these vessels. Women are disproportionately more affected than men by the disease, and if left untreated average life expectancy for some types of PAH can be as little as two to three years.