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. 2024 Apr 2;63(4):1139-1146.
doi: 10.1093/rheumatology/kead360.

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

Oliver Distler  1 , Christian Ofner  1 , Dörte Huscher  2 , Suzana Jordan  1 , Silvia Ulrich  3 , Gerd Stähler  4 , Ekkehard Grünig  5 , Matthias Held  6 , H Ardeschir Ghofrani  7 , Martin Claussen  8 , Tobias J Lange  9 , Hans Klose  10 , Stephan Rosenkranz  11 , Anton Vonk-Noordegraaf  12 , C Dario Vizza  13 , Marion Delcroix  14 , Christian Opitz  15 , Christine Pausch  16 , Laura Scelsi  17 , Claus Neurohr  18 , Karen M Olsson  19   20 , J Gerry Coghlan  21 , Michael Halank  22 , Dirk Skowasch  23 , Jürgen Behr  24 , Katrin Milger  24 , Bjoern Andrew Remppis  25 , Andris Skride  26 , Elena Jureviciene  27 , Lina Gumbiene  27 , Skaidrius Miliauskas  28 , Judith Löffler-Ragg  29 , Heinrike Wilkens  30 , David Pittrow  16   31 , Marius M Hoeper  19   20 , Ralf Ewert  32
Affiliations

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

Oliver Distler et al. Rheumatology (Oxford). .

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.

Keywords: connective tissue disease (CTD); endothelin receptor antagonists (ERA); phosphodiesterase type 5 inhibitor (PDE5i); pulmonary arterial hypertension; systemic sclerosis (SSc).

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Figures

Figure 1.
Figure 1.
Flow chart of the selection process for the study cohort
Figure 2.
Figure 2.
Kaplan–Meier survival estimates in various forms of CTD-PAH
Figure 3.
Figure 3.
Kaplan–Meier survival estimates in patients with SSc-PAH according to the initial treatment regimen
See this image and copyright information in PMC

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References

    1. Thakkar V, Lau EM.. Connective tissue disease-related pulmonary arterial hypertension. Best Pract Res Clin Rheumatol 2016;30:22–38. - PubMed
    1. Galie N, Humbert M, Vachiery JL. et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015;46:903–75. - PubMed
    1. Steen VD, Medsger TA.. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 2007;66:940–4. - PMC - PubMed
    1. Gurubhagavatula I, Palevsky HI.. Pulmonary hypertension in systemic autoimmune disease. Rheum Dis Clin North Am 1997;23:365–94. - PubMed
    1. Callejas-Rubio JL, Moreno-Escobar E, de la Fuente PM. et al. Prevalence of exercise pulmonary arterial hypertension in scleroderma. J Rheumatol 2008;35:1812–6. - PubMed

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