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Selexipag for the Treatment of Connective Tissue Disease-Associated PAH
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This program reviews the recent publication by Gaine et al (Eur Respir J, 2017) regarding use of the novel prostacyclin receptor agonist, selexipag, in patients with connective tissue disease related pulmonary hypertension. This review, by paper co-author Dr. Richard Channick examines the essential efficacy and safety of this drug in a group of patients considered hard-to-treat.

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Hello. My name is Richard Channick. I am at Massachusetts General Hospital in Boston. Today, I'd like to talk about a recently published paper looking at selexipag in patients with connective tissue disease associated pulmonary hypertension.

This paper was published recently in the European Respiratory Journal. We know that connective tissue disease is a particularly challenging form of pulmonary hypertension to treat. These patients have a high morbidity and mortality and it's important that we look at some of our affective PAH Therapies in this sub-group.
We had the opportunity to do that in the GRIPHON trial, which looked at long-term effects of selexipag, a Prostacyclin receptor agonist in patents with pulmonary arterial hypertension.

This was a study that looked at titrating this oral Prostacyclin agonist to maximally tolerated dose using a morbidity and mortality composite end-point as the primary end-point.

Within the overall study group of 1,156 patients, 334 of them had connective tissue disease associated PAH. The majority being scleroderma, but a significant number of patients with systemic lupus, mixed connective tissue disease, and other forms of connective tissue disease.

Patients who are in the connective disease sub-group ended up on low, medium, as well as high-dose of selexipag. The majority of these patients were female with a mean age in the fifties. And patients with scleroderma tended to be older.

The breakdown in terms of functional class and background therapy in these patients were so much similar to what we saw in the overall group that was studied. The majority of patients were in fact on background therapies.

What was found in this study was that overall there was a significant treatment effect of selexipag in benefiting patients who had connective tissue diseases associated PH. And that treatment effect was irrespective of whether the patient was on background therapy or not. In other words, even patients on an endothelin receptor antagonist, a PD5 inhibitor or even both had a similar treatment effect to the overall treatment effect.

In addition, it was shown in this analysis that regardless of which connective tissue disease was present, the benefit was seen equally with selexipag. And the Compton-Myers curves confirm that regardless of the cause of pulmonary hypertension which connective tissue we're dealing with, scleroderma or lupus, one saw similar benefits.

Adverse events were also mirroring what we typically see with prostacyclin-type drugs with headache, diarrhea, nausea, jaw pain, extremity pain being more common in the treated group versus placebo. Not at all surprising. There did not appear to be any greater degree or severity of adverse events in the connective tissue disease sub-group.

From this analysis, we conclude that in patients with connective tissue disease associated PH, the overall treatment effect of selexipag was similar to the overall GRIPHON population and irrespective of whether or not the patient was on background therapy or the sub-type of connective tissue disease. The composite end-point of morbidity and mortality was driven predominantly by reduction in disease progression and hospitalization.

In addition, there were some benefits of a six-minute walk distance that were similar to what's observed in the overall population. The drug was well tolerated in this group. And I think the data convincingly support the concept that multiple PAH therapies will benefit patients with connective tissue disease pulmonary hypertension, a group that historically has been difficult to treat.

Thank you for your attention.