Selexipag treatment in patients with systemic sclerosis-associated pulmonary arterial hypertension in clinical practice, a case series.
Jacqueline M J LemmersHåvard FretheimHanneke Ka KnaapenFrank Hj van den HoogenJolanda Hgm van Haren-WillemsAnthony L DuijnhouwerArie P van DijkCornelia Hm van den EndeAnna-Maria Hoffmann-VoldMadelon C VonkPublished in: Journal of scleroderma and related disorders (2020)
Adding selexipag to background therapy in a high-risk cohort of systemic sclerosis-associated pulmonary arterial hypertension patients provided sustained stabilization of symptoms with an acceptable safety profile. Improvement was reached in only two of our patients. Further research should focus on systemic sclerosis-associated pulmonary arterial hypertension patients treated with multiple targeted treatments, preferably these patients should be prospectively followed in international registries.