Eye signs as a novel risk predictor in pulmonary arterial hypertension associated with systemic lupus erythematosus

Abstract Objective To investigate the role of eye signs in predicting poor outcome of systemic lupus erythematosus (SLE) patients with pulmonary arterial hypertension (PAH). Methods This prospective observational study recruited the patients diagnosed with SLE-PAH from Jan. 2010 to Dec. 2010 at the first affiliated hospital of Nanchang University, while those with other potential causes of PAH were excluded. the evaluation of various parameters such as N-terminal prohormone of brain natriuretic peptide (NT-proBNP), 6-minute walking distance(6MWD), World Health Organization functional class (WHO-FC), echocardiography, and risk stratification based on the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) Guidelines were conducted at intervals of every 1–3 months, and a 6-month follow-up period was observed. The primary outcome measure considered improvement if there was a decline in the risk stratification grade at the end point, and unimproved if there was no decline. Conjunctival microvasculation images were observed and recorded. Results A total of 29 SLE-PAH patients were enrolled, comprising 12 in the improved group and 17 in the non-improved group. ALL SLE-PAH show various manifestions in eye signs including vessel twisting, dilation, ischemic areas, hemorrhages, reticulum deformity, and wound spots. The non-improved group exhibited significantly lower vessel density (VD) and microvascular flow index (MFI) of conjuctival microvasculation images compared to the improved group. Correlation analysis revealed that VD displayed a negative correlation with the WHO-FC(r=-0.413, p = 0.026)and NT-proBNP (r=-0.472, p = 0.010), as well as a positive correlation with the 6MWD(r = 0.561, p = 0.002). Similarly, MFI exhibited a negative correlation with WHO-FC (r=-0.408, p = 0.028), and NT-proBNP (r=-0.472, p = 0.010), and a positive correlation with 6MWD (r = 0.157, p = 0.004). Multivariate logistic regression analysis indicated that VD (OR 10.11, 95% CI 1.95–52.36), MFI (OR 7.85, 95% CI 1.73–35.67), NT-proBNP, and 6MWD were influential factors in predicting the prognostic improvement of SLE-PAH patients. ROC curve analysis demonstrated that VD, MFI, 6MWD, and NT-proBNP (with respective ROC AUC values of 0.83, 0.83, 0.76, and 0.90) possessed a sensitivity and specificity of 75% and 100%, as well as 83% and 100%, respectively. Regarding prognostic prediction, VD and MFI exhibited higher sensitivity compared to 6MWD, whereas MFI displayed higher sensitivity and specificity compared to NT-proBNP. Conclusion SLE-PAH can lead to various conjuctival microvascular manifestions in which vascular density and microvascular flow index can be used to assess the cardiopulmonary function and predict therapeutic efficacy and prognosis in SLE-PAH patients.