Risk stratification for systemic lupus erythematosus associated pulmonary hypertension by simplified ESC/ERS guidelines: data from SOPHIE registry

Abstract

Abstract Background The ESC/ERS guideline has established a diagnostic algorithm for pulmonary hypertension (PH) based on the peak velocity of tricuspid regurgitation, clinical history of left heart disease, chest radiography, and other chest radiological techniques. Unfortunately, due to the medical resource limitations, not all patients can receive the comprehensive inspections listed above. Purpose The purpose of the study was to sort out the practical predicting factors and evaluate the efficacy of the simplified diagnostic algorithm derived from the 2015 ESC/ERS clinical guidelines. Methods According to the ESC/ERS guideline, patients in the intermediate or high echocardiographic probability of PH group and further satisfied the following risk stratification criteria (anti-RNP (+), Raynaud phenomenon (+), 6MWD <440 meters, and SVR >1500 wood) were considered to have the intermediate (IR), and high risk (HR) of PH. Patients with a low echocardiographic probability of PH and those with intermediate or high probability of PH without satisfied the risk stratification algorithm were categorized into the low risk (LR) of the PH group. Results 1005 patients were categorized into the LR group, 30 patients in the IR group, and 19 in the HR group. Precisely, patients in the LR group were relatively younger (49.9±0.4) than that of the IR group (57.8±2.3, p=0.001) and the HR group (56.0±3.0, p=0.035). Raynaud phenomenon was occurred more frequently in the IR group (50.0%) or HR group (47.4%) compared to that of the LR group (24.1%). The occurrence of anti-RNP positive was higher in the HR group, taken up for 57.9% compared to 31.5% in the LR group (p=0.013). The incidence of right axis deviation (RAD) was increasing in the IR group (17.2%) and HR group (16.7%) compared to the LR group (3.6%). In addition, patients in IR and HR group have reduced lung volume parameters, including forced vital capacity (FVC%), forced expiratory volume at the first second (FEV1%), FEV1/FVC ratio, and forced expiratory flow at 50% of forced vital capacity (FEF50%) when compared to the LR group. Multivariate analysis was performed in four different modes to sort out the independent predictors of PH. Anti-RNP (OR=10.5, CI: 1.9, 56.5) and RAD (OR=21.9, CI: 5.2, 92.2) in mode 1, RVH (OR=10.4, CI: 1.9, 56.3) in mode 2, RAD (OR=10.3, CI: 2.0, 50.7) and FEF50% (OR=1.0, CI: 0.90, 0.97) in mode 3, and RVH (OR=56.8, CI: 6.9, 467.5) and FEF50% (OR=1.0, CI: 0.90, 0.97) in mode 4 were identified as the independent predictors of PH for SLE patients. Conclusions This registry has provided main clinical characteristics and phenotypes of Hong Kong Chinese patients with SLE and has demonstrated that Anti-RNP, RAD, RVH, or FEF50% was independent predictors of pulmonary hypertension in patients with SLE. Funding Acknowledgement Type of funding sources: None.