학술논문
Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis.
Document Type
Article
Author
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
Source
Subject
*COMBINATION drug therapy
*SURVIVAL rate
*RESEARCH funding
*LONG-term health care
*FISHER exact test
*TREATMENT effectiveness
*DESCRIPTIVE statistics
*SYSTEMIC lupus erythematosus
*MANN Whitney U Test
*CHI-squared test
*CONNECTIVE tissue diseases
*PHOSPHODIESTERASE inhibitors
*SYSTEMIC scleroderma
*PULMONARY arterial hypertension
*COMPARATIVE studies
*DATA analysis software
*ENDOTHELINS
*CELL receptors
*CHEMICAL inhibitors
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Language
ISSN
1462-0324
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. [ABSTRACT FROM AUTHOR]