Survival and prognosis analysis of systemic lupus erythematosus patients with pulmonary hypertension: A systematic review and meta-analysis

BACKGROUND: The study aimed to evaluate survival rates and prognosis in systemic lupus erythematosus (SLE) patients with pulmonary hypertension (PH) using meta-analysis. METHODS: PubMed, EMBASE, Cochrane central register of controlled trials, China National Knowledge Infrastructure, China Science and Technology Journal Database, Wan-Fang Database, and Chinese biomedical database were searched. Information and data were screened and extracted by 2 researchers. The obtained data were analyzed using the R software meta package. Quality assessment was conducted using Newcastle-Ottawa Scale. The causes of heterogeneity were analyzed using subgroup analysis and sensitivity analysis. Publication bias was evaluated using Begger funnel plots and Egger test. RESULTS: The search strategy yielded a total of 21 studies involving 875 patients included in the final analysis. The pooled 1-, 3- and 5-year survival rates of patients with SLE-PH were 0.9020 (95%CI: 0.8576; 0.9397), 0.8363 (96%CI: 0.7813; 0.8852), 0.7301 (95%CI: 0.6327; 0.8181). The 1-, 3- and 5-year survival rates of echocardiography subgroup were 0.9000 (95%CI: 0.8480; 0.9551), 0.8435 (95%CI: 0.7744; 0.9187), 0.6795 (95%CI: 0.5746; 0.8035), respectively; and there were 0.9174 (95%CI: 0.8951; 0.9402), 0.8529 (95%CI: 0.8255; 0.8812), 0.7757 (95%CI: 0.7409; 0.8121) at right heart catheterization subgroup in the meantime. Multivariate analysis for predicting mortality in SLE-PH patients revealed that diminishing left ventricular ejection fraction, New York Heart Association classification, lupus nephritis, lower cardiac index, and higher red blood cell distribution width level were significantly associated with a higher mortality rate. Treatment with huge doses of cyclophosphamide, tricuspid annular plane systolic excursion/pulmonary artery systolic pressure, and Raynaud phenomenon signaled favorable outcomes. CONCLUSION: The 1-, 3-, and 5-year survival rates of SLE-PH patients in recent years (0.9020, 0.8363, 0.7301) were estimated in this study. SLE-PH patients diagnosed by echocardiography have a worse long-term prognosis than those diagnosed by right heart catheterization. Studies after 2015 have shown significantly better survival than earlier studies.