Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis - Forschungsportal der Justus-Liebig-Universität Gießen:

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Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis

Autorenliste: Distler, Oliver; Ofner, Christian; Huscher, Doerte; Jordan, Suzana; Ulrich, Silvia; Staehler, Gerd; Gruenig, 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, Juergen; Milger, Katrin; Remppis, Bjoern Andrew; Skride, Andris; Jureviciene, Elena; Gumbiene, Lina; Miliauskas, Skaidrius; Loeffler-Ragg, Judith; Wilkens, Heinrike; Pittrow, David; Hoeper, Marius M.; Ewert, Ralf

Jahr der Veröffentlichung: 2024

Seiten: 1139-1146

Zeitschrift: Rheumatology

Bandnummer: 63

Heftnummer: 4

ISSN: 1462-0324

eISSN: 1462-0332

DOI Link: https://doi.org/10.1093/rheumatology/kead360

Verlag: Oxford University Press

Abstract:
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.

Zitierstile

Harvard-Zitierstil: Distler, O., Ofner, C., Huscher, D., Jordan, S., Ulrich, S., Staehler, G., et al. (2024) Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis, Rheumatology, 63(4), pp. 1139-1146. https://doi.org/10.1093/rheumatology/kead360

APA-Zitierstil: Distler, O., Ofner, C., Huscher, D., Jordan, S., Ulrich, S., Staehler, G., Gruenig, E., Held, M., Ghofrani, H., Claussen, M., Lange, T., Klose, H., Rosenkranz, S., Vonk-Noordegraaf, A., Vizza, C., Delcroix, M., Opitz, C., Pausch, C., Scelsi, L., ...Ewert, R. (2024). Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis. Rheumatology. 63(4), 1139-1146. https://doi.org/10.1093/rheumatology/kead360

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