• Eric Hachulla, David Launay, Athénaïs Boucly, Luc Mouthon, Pascal de Groote, Vincent Cottin, Grégory Pugnet, Grégoire Prévôt, Delphine Bourlier, Claire Dauphin, Ari Chaouat, Jason Weatherald, Gérald Simonneau, David Montani, Marc Humbert, Olivier Sitbon, and Jonathan Giovannelli.
• Department of Internal Medicine and Clinical Immunology, Claude Huriez Hospital, Referral Center for Rare Systemic Autoimmune Diseases North and Northwest of France, Lille, France; Université de Lille, Inserm, CHU Lille, U995-LIRIC-Lille Inflammation Research International Center, F-59000 Lille, France. Electronic address: eric.hachulla@chru-lille.fr.
• Chest. 2020 Apr 1; 157 (4): 945-954.

BackgroundTo date, nothing is known about the evolution of survival in systemic sclerosis-associated pulmonary arterial hypertension (PAH) over the last decade.MethodsThis study used a multivariate Cox regression model adjusted for clinically relevant baseline confounders to assess the association between the occurrence of death and date of PAH diagnosis comparing two periods of the same duration (2006-2011 vs 2012-2017). Interactions between the two diagnosis periods and baseline variables were tested.ResultsA total of 306 incident patients were included, 167 (54.6%) with a PAH diagnosis occurring in 2006 to 2011 and 139 (45.4%) in 2012 to 2017. No significant difference in survival was observed between patients diagnosed with PAH in 2012 to 2017 compared with those diagnosed in 2006 to 2011 (hazard ratio [HR], 0.76 [0.46-1.26]; P = .29). A significant interaction was observed between PAH diagnosis periods and age (P = .05). When stratifying according to age (based on the median age of 70 years), a significant increase was observed in survival in patients aged ≤ 70 years between the 2006 to 2011 period and the 2012 to 2017 period (HR, 0.40 [0.17-0.99]; P = .046) but not in older patients (HR, 1.29 [0.67-2.51]; P = .44). A significantly higher proportion of initial (ie, within the first 4 months) endothelin receptor antagonist/phosphodiesterase type 5 inhibitor combination therapy was observed in younger patients diagnosed from 2012 to 2017 vs those diagnosed from 2006 to 2011 (42.9% vs 19.5%; P = .002) but not in older patients.ConclusionsOver the period 2006 to 2017, survival in systemic sclerosis-associated PAH improved over time in patients aged ≤ 70 years but not in older patients. Further investigations are needed to confirm this relation, as general improvement in medical care and management may also be a possible explanation.Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.

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