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

Abstract

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.

Figure 1.

Flow chart of study selection process.

Figure 2.

Forest plot of 1-, 3-, and 5-yr survival rates.