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