Initial treatment strategies and outcomes across haemodynamic thresholds and risk stratification in incident systemic sclerosis-associated pulmonary arterial hypertension: a EUSTAR analysis.
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Bjørkekjær Hilde Jenssen, Bruni Cosimo, Broch Kaspar, Brunborg Cathrine, Carreira Patricia E, Airò Paolo, Simeón-Aznar Carmen Pilar, Truchetet Marie-Elise, Giollo Alessandro, Balbir-Gurman Alexandra, Martin Mickael, Denton Christopher P, Gabrielli Armando, Del Galdo Francesco, Vonk Madelon C, Fretheim Håvard, Bitter Helle, Midtvedt Øyvind, Andreassen Arne K, Høie Sverre, Tanaka Yoshiya, Riemekasten Gabriela, Müller-Ladner Ulf, Matucci-Cerinic Marco, Castellví Ivan, Siegert Elise, Hachulla Eric, Molberg Øyvind, Distler Oliver, Hoffmann-Vold Anna-Maria, EUSTAR collaborators
Аннотация
METHODS: We analysed treatment-naïve SSc-PAH patients in EUSTAR (2000-2021) fulfilling the 2022 haemodynamic definition. Initial therapy comprised treatment initiated within 4 months of RHC. As-treated (AT) analyses were primary and intention-to-treat (ITT) analyses supportive. Outcomes were all-cause mortality and 36-month PAH progression.
RESULTS: Among 301 patients, 198 (66%) initiated therapy and 170 maintained therapy for ≥3 months. Therapy was initiated in 31% with milder haemodynamic impairment (mPAP 21-24 mmHg and/or PVR 2-3 WU) versus 79% fulfilling the previous haemodynamic definition; combination therapy increased from 14% to 67% across low- to high-risk strata. Over a median 3.9 years, 125 (42%) died and 85/227 (37%) progressed. Adjusted ITT analyses showed no association with either outcome, whereas adjusted AT analyses showed lower mortality risk with sustained monotherapy and combination therapy (HR 0.50, 95% CI 0.27-0.92 and HR 0.40, 95% CI 0.18-0.88, respectively) and lower PAH progression risk with sustained initial therapy (HR 0.37, 95% CI 0.18-0.76). In the milder haemodynamic subgroup, the mortality estimate was directionally consistent but imprecise.
CONCLUSION: Initial treatment strategies reflected haemodynamic severity and baseline risk, while variation suggested potential implementation gaps. Sustained initial therapy was associated with lower risks of mortality and PAH progression. Contrasting ITT and AT estimates highlight challenges in observational treatment-outcome comparisons. Findings in the milder haemodynamic subgroup support dedicated prospective studies.