Pulmonary hypertension in systemic lupus erythematosus: echocardiography-based definitions predict 6-year survival

Rheumatology (Oxford). 2014 Jul;53(7):1256-63. doi: 10.1093/rheumatology/keu012. Epub 2014 Mar 5.


Objective: The aim of this study was to investigate whether a core of echocardiography-based definitions of pulmonary hypertension (PH), as proposed by the European Society of Cardiology, European Respiratory Society and International Society of Heart and Lung Transplantation (ESC/ERS/ISHLT), may predict long-term survival in patients with SLE.

Methods: A post hoc analysis from a cohort of SLE patients followed over 6 years was performed. Clinical associations, serum biomarkers, autoantibody profile, length of survival and all-cause mortality were assessed.

Results: Out of 115 patients from the original cohort, 55 satisfied our inclusion criteria and were grouped according to echocardiography as unlikely (n = 26), possible (n = 16) or likely (n = 13) to have PH. Likely PH was associated with a history of pulmonary thromboembolism, higher cumulated organ damage and active arthritis. The 6-year survival rate was 88% in the unlikely PH group, 87% in the possible PH group and 68% in the likely PH group (P < 0.05). Serum levels of endothelin-1, monocyte chemotactic protein-1, IL-17, and IFN-γ as well as a number of autoantibodies were no different between groups.

Conclusion: The ESC/ERS/ISHLT echocardiography-based definitions of PH are useful to predict 6-year mortality in SLE patients. A history of pulmonary thromboembolism and lung vasculitis/haemorrhage, cumulated organ damage and long-lasting disease are associated with PH in SLE.

Keywords: MCP-1; echocardiography; endothelin-1; pulmonary hypertension; survival; systemic lupus erythematosus.

MeSH terms

  • Adult
  • Cohort Studies
  • Comorbidity
  • Echocardiography*
  • Female
  • Follow-Up Studies
  • Hemorrhage / complications
  • Humans
  • Hypertension, Pulmonary / diagnostic imaging*
  • Hypertension, Pulmonary / epidemiology*
  • Longitudinal Studies
  • Lupus Erythematosus, Systemic / epidemiology*
  • Lupus Erythematosus, Systemic / mortality*
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Pulmonary Embolism / complications
  • Risk Factors
  • Survival Rate
  • Time Factors
  • Vasculitis / complications