Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: A COMPERA analysis.
Oliver DistlerChristian OfnerDörte HuscherSuzana JordanSilvia UlrichGerd StählerEkkehard GrünigMatthias HeldH Ardeschir GhofraniMartin ClaussenTobias J LangeHans KloseStephan RosenkranzAnton Vonk-NoordegraafC Dario VizzaMarion DelcroixChristian OpitzChristine PauschLaura ScelsiClaus NeurohrKaren M OlssonJ Gerry CoghlanMichael HalankDirk SkowaschJürgen BehrKatrin MilgerBjoern Andrew RemppisAndris SkrideElena JurevicieneLina GumbieneSkaidrius MiliauskasJudith Löffler-RaggHeinrike WilkensDavid PittrowMarius M HoeperRalf EwertPublished in: Rheumatology (Oxford, England) (2023)
Mortality remains high in patients with CTD-PAH, especially for patients with SSc-PAH. However, for patients with SSc-PAH, our results suggest that long-term survival may be improved with initial ERA/PDE5i combination therapy compared with initial monotherapy.