Selexipag Delays PAH Progression in Patients with Connective Tissue Disease
Results of a subgroup analysis from the GRIPHON trial are promising for a hard-to-treat population.
Pulmonary arterial hypertension (PAH) is a dangerous complication of connective tissue disease (CTD), eg, systemic sclerosis, systemic lupus erythematosus, and has been historically difficult to treat. Recent studies suggest that treatment regimens that combine PAH therapies may be more effective in this population, but the studies are few. The current study analyzed a subgroup of patients with PAH-CTD enrolled in the GRIPHON trial (selective IP prostacyclin receptor agonist selexipag) to assess the impact of selexipag on PAH progression across CTD types. The study and results are summarized in this short slide show.
Selexipag Delays PAH Progression in Patients with Connective Tissue Disease
GRIPHON: Improved PAH Outcomes with Selexipag. In a double-blind, randomized, placebo controlled trial, SLXPG decreased morbidity and mortality by 40% vs placebo.
Does Response Vary by CTD Subtype? The GRIPHON study had the largest number of PAH-CTD patients evaluated to date in a double-blind RCT.
Delayed PAH Progression Seen Across CTD Subtypes. Overall PAH-CTD had 41% lower risk of primary endpoint with SLXPG.
Selexipag Well-Tolerated in PAH-CTD. Most commonly reported adverse events included headache, diarrhea, nausea, dizziness, and jaw pain.
Study Limitations Included:
- PAH-CTD subtypes were not pre-specified subgroups in the larger study.
- Investigator identified CTD subtype - no other disease specific information or serologies available.
- Trial was not designed to evaluate mortality.
Potential Strategy for Difficult-to-Treat Patients. ". . . these data support the use of multiple PAH therapies when treating patients with PAH-CTD and emphasise that this treatment strategy can yield benefits." First author Sean Gaine said.
Take-home Points:
- Long-term results from GRIPHON trial found SLXPG delayed progression of PAH among patients with PAH-CTD regardless of CTD subtypes and baseline therapy.
- Tolerability was similar to the general GRIPHON population.
- SLXPG may offer incremental benefit in combination therapy for PAH-CTD.
