Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: A COMPERA analysis
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Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: A COMPERA analysis. / Distler, Oliver; Ofner, Christian; Huscher, Dörte; Jordan, Suzana; Ulrich, Silvia; Stähler, Gerd; Grünig, Ekkehard; Held, Matthias; Ghofrani, H Ardeschir; Claussen, Martin; Lange, Tobias J; Klose, Hans; Rosenkranz, Stephan; Vonk-Noordegraaf, Anton; Vizza, C Dario; Delcroix, Marion; Opitz, Christian; Pausch, Christine; Scelsi, Laura; Neurohr, Claus; Olsson, Karen M; Coghlan, J Gerry; Halank, Michael; Skowasch, Dirk; Behr, Jürgen; Milger, Katrin; Remppis, Bjoern Andrew; Skride, Andris; Jureviciene, Elena; Gumbiene, Lina; Miliauskas, Skaidrius; Löffler-Ragg, Judith; Wilkens, Heinrike; Pittrow, David; Hoeper, Marius M; Ewert, Ralf.
in: RHEUMATOLOGY, Jahrgang 63, Nr. 4, 02.04.2024, S. 1139-1146.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: A COMPERA analysis
AU - Distler, Oliver
AU - Ofner, Christian
AU - Huscher, Dörte
AU - Jordan, Suzana
AU - Ulrich, Silvia
AU - Stähler, Gerd
AU - Grünig, Ekkehard
AU - Held, Matthias
AU - Ghofrani, H Ardeschir
AU - Claussen, Martin
AU - Lange, Tobias J
AU - Klose, Hans
AU - Rosenkranz, Stephan
AU - Vonk-Noordegraaf, Anton
AU - Vizza, C Dario
AU - Delcroix, Marion
AU - Opitz, Christian
AU - Pausch, Christine
AU - Scelsi, Laura
AU - Neurohr, Claus
AU - Olsson, Karen M
AU - Coghlan, J Gerry
AU - Halank, Michael
AU - Skowasch, Dirk
AU - Behr, Jürgen
AU - Milger, Katrin
AU - Remppis, Bjoern Andrew
AU - Skride, Andris
AU - Jureviciene, Elena
AU - Gumbiene, Lina
AU - Miliauskas, Skaidrius
AU - Löffler-Ragg, Judith
AU - Wilkens, Heinrike
AU - Pittrow, David
AU - Hoeper, Marius M
AU - Ewert, Ralf
N1 - © The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.
PY - 2024/4/2
Y1 - 2024/4/2
N2 - OBJECTIVES: Pulmonary arterial hypertension (PAH) occurs in various connective tissue diseases (CTDs). We sought to assess contemporary treatment patterns and survival of patients with various forms of CTD-PAH.METHODS: We analysed data from COMPERA, a European pulmonary hypertension registry, to describe treatment strategies and survival in patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types of CTD. All-cause mortality was analysed according to the underlying CTD. For patients with SSc-PAH, we also assessed survival according to initial therapy with endothelin receptor antagonists (ERAs), phosphodiesterase type 5 inhibitors (PDE5is) or a combination of these two drug classes.RESULTS: This analysis included 607 patients with CTD-PAH. Survival estimates at 1, 3 and 5 years for SSc-PAH (n = 390) were 85%, 59% and 42%; for SLE-PAH (n = 34) they were 97%, 77% and 61%; for MCTD-PAH (n = 33) they were 97%, 70% and 59%; for UCTD-PAH (n = 60) they were 88%, 67% and 52%; and for other CTD-PAH (n = 90) they were 92%, 69% and 55%, respectively. After multivariable adjustment, the survival of patients with SSc-PAH was significantly worse compared with the other conditions (P = 0.001). In these patients, the survival estimates were significantly better with initial ERA-PDE5i combination therapy than with initial ERA or PDE5i monotherapy (P = 0.016 and P = 0.012, respectively).CONCLUSIONS: 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.
AB - OBJECTIVES: Pulmonary arterial hypertension (PAH) occurs in various connective tissue diseases (CTDs). We sought to assess contemporary treatment patterns and survival of patients with various forms of CTD-PAH.METHODS: We analysed data from COMPERA, a European pulmonary hypertension registry, to describe treatment strategies and survival in patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types of CTD. All-cause mortality was analysed according to the underlying CTD. For patients with SSc-PAH, we also assessed survival according to initial therapy with endothelin receptor antagonists (ERAs), phosphodiesterase type 5 inhibitors (PDE5is) or a combination of these two drug classes.RESULTS: This analysis included 607 patients with CTD-PAH. Survival estimates at 1, 3 and 5 years for SSc-PAH (n = 390) were 85%, 59% and 42%; for SLE-PAH (n = 34) they were 97%, 77% and 61%; for MCTD-PAH (n = 33) they were 97%, 70% and 59%; for UCTD-PAH (n = 60) they were 88%, 67% and 52%; and for other CTD-PAH (n = 90) they were 92%, 69% and 55%, respectively. After multivariable adjustment, the survival of patients with SSc-PAH was significantly worse compared with the other conditions (P = 0.001). In these patients, the survival estimates were significantly better with initial ERA-PDE5i combination therapy than with initial ERA or PDE5i monotherapy (P = 0.016 and P = 0.012, respectively).CONCLUSIONS: 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.
U2 - 10.1093/rheumatology/kead360
DO - 10.1093/rheumatology/kead360
M3 - SCORING: Journal article
C2 - 37462520
VL - 63
SP - 1139
EP - 1146
JO - RHEUMATOLOGY
JF - RHEUMATOLOGY
SN - 1462-0324
IS - 4
ER -
