Objective: Pulmonary arterial hypertension (PH) is a progressive disease with a poor prognosis that ultimately leads to right ventricular failure and death. The pathogenesis of severe PH seems to be related to inflammatory responses and coagulation disturbances. Many diseases can develop PH in their course, thus aggravating their outcome. The objective was to investigate the values of vascular endothelial growth factor (VEGF), sP-selectin, lipoprotein-associated phospholipase A2 (PLA2-LDL), antiphospholipid antibodies (APLA) and their relation with PH, in systemic lupus erythematosus (SLE) and chronic obstructive pulmonary disease (COPD), two conditions in which the occurrence of PH is frequent.
Design: Prospective clinical study.
Setting: A University Department of Internal Medicine, a National Institute of Research.
Patients: 30 SLE patients (15 patients without PH (group I) and 15 patients with PH group II)), 30 patients with COPD (15 patients without PH (group III) and 15 patients with PH (group IV)) and 10 healthy controls, selected by clinical, immunological, echocardiographical criteria and pulmonary functional tests.
Main outcome measures: VEGF, sP-selectin and PLA2-LDL level in plasma and presence of antiphospholipids antibodies (lupus anticoagulant, anticardiolipin and anti beta2 GPI) in plasma. -
Results: In patients with PH, the values of VEGF were significantly increased [group II (1023.1) and IV (904.3)] compared with group I (744.2), III (356.4), and controls (330.3). The values of sP-selectin in group II (9.7), and IV (10.4) were also increased compared with controls (6). APLA were present in all patients in group II (100%), and in 8 patients in group IV (53%), while in the other groups the frequency was low (33% group I and 13% group III). PLA2-LDL activity was higher in group II (429.1) and group IV (394.5) than in group I (317.8), group III (343.2) and controls (256.3).
Conclusion: PH is a severe complication in COPD and SLE. The increased values of VEGF, PLA2-LDL and P-selectin in patients with long standing PH are related to severe endothelial dysfunction and may have prognostic values. APLA may have pathogenic value in SLE patients with PH. APLA are possibly implicated in the pathogenesis of PH in these diseases. VEGF, APLA and sP-selectin may constitute new therapeutic targets for PH.