| Objective This study was to investigate the clinical characteristics,risk factors and prognostic factors of Systemic lupus Erythematosus(SLE)patients with Pulmonary arterial hypertension(PAH),and to provide evidence for diagnosis and treatment of SLE-PAH.Methods This study retrospectively collected 3888 patients with systemic lupus erythematosus(SLE)and 158 patients with SLE-PAH who were hospitalized in the First Affiliated Hospital of Guangxi Medical Universityfrom January 1,2012 to January 1,2018.240 SLE-non PAH patients matched by gender and age were randomly selected from 3730 SLE-non PAH patients,The general data,clinical characteristics,autoantibody type,inflammation index and system involvement of the two groups were compared.Patients in SLE-PAH group were divided into who-fc grade I/II cardiac function group and Ⅲ/Ⅳ cardiac function group,After treatment with glucocorticoid and immunosuppressant,patients were divided into death group and survival group according to their prognosis.Logistic regression analysis was used to analyze the related factors and prognostic risk factors.Results 1.In this study,the proportion of SLE-PAH in SLE patients was 4.1%(158/3888 cases).15.8%(25/158 cases)of SLE-PAH patients had hypertension,Compared with SLE-non PAH group,there were less photosensitivity,rash and antinuclear antibody nucleoli in SLE-PAH group(P<0.05),Raynaud’s phenomenon,fever,edema,cough and expectoration,chest distress and chest pain,dyspnea were more common(P<0.05),systemic lupus erythematosus activity index score(SLEDAI)increased significantly,thrombocytopenia,hemolytic anemia,interstitial lung disease(ILD),pericardial effusion,neuropsychiatric lupus erythematosus(NPSLE),lupus nephritis(LN),anti La/SSB antibody positive,anti ds-DNA antibody quantitative,ESR,RF,Ig G,NT-pro BNP increased,C3 and C4 decreased more frequently in SLE-PAH group(P<0.05).2.In SLE-PAH patients,compared with the patients of grade I/II of cardiac function,the positive rate of anti Ro-52 antibody increased,cough and expectoration,chest distress and chest pain,dyspnea,lung involvement and ILD were more common in the group of Ⅲ/Ⅳ of cardiac function,PASP increased,right cardiac enlargement,main pulmonary artery widened,and a large amount of pericardial effusion were more common and arthritis is rare(P<0.05).3.158 SLE-PAH patients were followed up for more than 1 year,29 cases died,nine patients died of severe infection directly(3 cases with NPSLE,1 case with right heart failure),one case died of sudden cardiac death caused by severe PAH.Compared with the survival group,the death group had dyspnea(68.9% vs47.1%),thrombocytopenia(34.5% vs 17.6%),heart function grade Ⅲ/Ⅳ(68.9% vs 30.6%),anti Ro-52 antibody positive rate(72.4% vs 46.3%),NT-pro BNP increased significantly,PASP increased and right ventricular enlargement were more common in echocardiography(P<0.05),homogeneous type of anti nuclear antibody is rare.The anti ds-DNA antibody in the survival group increased significantly(P<0.05).4.The 1-year,3-year and 5-year survival rates of SLE-PAH patients were 91.5%,85.0% and 76.0%.5.Multivariate logistic regression analysis,the independent risk factors of SLE-PAH were dyspnea,pericardial effusion,ILD,LN,anti La/SSB antibody positive,RF increased.Multivariate Cox regression analysis showed that the risk factors of death in SLE-PAH patients were thrombocytopenia and cardiac function Ⅲ/Ⅳ.Conclusions 1.In this study,the proportion of SLE-PAH in SLE patients was4.1%.In SLE-PAH patients,rash and photosensitivity are rare,and SLE patients with hypertension history,positive anti La/SSB antibody and high disease activity are more likely to have PAH.2.The independent risk factors of SLE-PAH were dyspnea,pericardial effusion,ILD,LN,anti La/SSB antibody positive,RF increased.The 1-year,3-year and5-year survival rates of SLE-PAH patients were 91.5%,85.0%,76.0%,and the risk factors of death were thrombocytopenia and grade Ⅲ/Ⅳ cardiac function. |
PDF Full Text Request