Science and Research

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

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.

  • Distler, O.
  • Ofner, C.
  • Huscher, D.
  • Jordan, S.
  • Ulrich, S.
  • Stähler, G.
  • Grünig, E.
  • Held, M.
  • Ghofrani, H. A.
  • Claussen, M.
  • Lange, T. J.
  • Klose, H.
  • Rosenkranz, S.
  • Vonk-Noordegraaf, A.
  • Vizza, C. D.
  • Delcroix, M.
  • Opitz, C.
  • Pausch, C.
  • Scelsi, L.
  • Neurohr, C.
  • Olsson, K. M.
  • Coghlan, J. G.
  • Halank, M.
  • Skowasch, D.
  • Behr, J.
  • Milger, K.
  • Remppis, B. A.
  • Skride, A.
  • Jureviciene, E.
  • Gumbiene, L.
  • Miliauskas, S.
  • Löffler-Ragg, J.
  • Wilkens, H.
  • Pittrow, D.
  • Hoeper, M. M.
  • Ewert, R.

Keywords

  • Humans
  • *Pulmonary Arterial Hypertension/etiology/complications
  • *Mixed Connective Tissue Disease/complications/drug therapy
  • *Connective Tissue Diseases/complications/drug therapy/diagnosis
  • *Hypertension, Pulmonary/drug therapy/etiology
  • Familial Primary Pulmonary Hypertension/complications
  • *Lupus Erythematosus, Systemic/complications/drug therapy
  • *Scleroderma, Systemic/complications
  • connective tissue disease (CTD)
  • endothelin receptor antagonists (ERA)
  • phosphodiesterase type 5 inhibitor (PDE5i)
  • pulmonary arterial hypertension
  • systemic sclerosis (SSc)
Publication details
DOI: 10.1093/rheumatology/kead360
Journal: Rheumatology (Oxford)
Pages: 1139-1146 
Number: 4
Work Type: Original
Location: Assoziierter Partner, ARCN, BREATH, CPC-M, TLRC, UGMLC
Disease Area: PH
Partner / Member: BIH, Ghd, JLU, KUM, MHH, Thorax
Access-Number: 37462520

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