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

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.