| Background and purposeSystemic lupus erythematosus(systemic lupus erythematosus,referred to as SLE)is an autoimmune-mediated diffuse connective tissue disease in women of childbearing age,which can involve many systems such as skin,joints,organs and so on.Pulmonary hypertension(pulmonary hypertension,PH)is a group of clinical syndrome characterized by increased pulmonary vascular resistance caused by different etiology and pathogenesis,which can increase the afterload of right ventricle and,in severe cases,lead to right heart dilatation and right heart failure with poor prognosis.Pulmonary arterial hypertension(PAH)is one of the common complications of connective tissue disease,and belongs to the category of pulmonary arterial hypertension(PH),in which the number of cases of SLE with PAH is the most,and PAH seriously affects the prognosis of SLE patients.The prevalence rate of SLE with PAH in China is about 3.8%,and the 5-year survival rate is 72.9%.The pathogenesis of SLE complicated with PAH is complex,which may be related to a variety of pathophysiological changes,such as pulmonary vascular remodeling,vascular endothelial dysfunction,immune complex-mediated pulmonary vasculitis,interstitial lung disease and microthrombosis.Patients with SLE complicated with PAH may show clinical manifestations such as progressive exertional dyspnea,palpitation,fatigue and impaired exercise tolerance,and delay diagnosis and treatment because the clinical manifestations are not specific and easy to be ignored.Early diagnosis and standard treatment are of great significance to the prognosis of SLE complicated with PAH.At present,the gold standard of PAH diagnosis is right cardiac catheterization,but its clinical application is limited because of its poor repeatability,invasiveness,high risk,high cost and so on.Transthoracic echocardiography is the first choice for SLE combined with PAH screening,which is not specific and easy to be affected by many factors in the early diagnosis of the disease,so it is of great significance to explore the early clinical markers.The purpose of this study is to explore the clinical characteristics of patients with SLE complicated with PAH through retrospective study,and predict risk factors,so as to provide objective basis for early identification of high-risk groups.Research objects and methodsThe patients with SLE who were enrolled in the first affiliated Hospital of Zhengzhou University from January 2019 to June 2020 were divided into PAH group and non-PAH group by transthoracic echocardiography.Inclusion criteria of all subjects:the diagnostic basis of 1SLE patients met the classification criteria of systemic lupus erythematosus in 1997 or 2012 or 2019.The definition of PAH is the pulmonary artery systolic pressure estimated by transthoracic echocardiography at rest is more than 36mmHg,and the tricuspid regurgitation velocity is higher than 2.8m/s.Exclusion criteria:age<18 years old,congenital heart disease,other connective tissue diseases with pulmonary hypertension,history of coronary heart disease or myocardial infarction,pulmonary embolism,severe valvular heart disease,severe bleeding and coagulation disorder,severe hepatic and renal insufficiency,pregnancy and so on.Through the implementation of the above inclusion and exclusion criteria,the demographic data,main clinical manifestations,laboratory and imaging data and SLE disease activity score(SLEDAI)were collected respectively.A unified Excel table was established for all patients’ clinical data,and SPSS 25 software was used for statistical analysis.The counting data were expressed by proportion and percentage,and the x2 test was used for comparison between groups.The measurement data that did not accord with normal distribution were expressed by M(P25～P75),and the comparison between groups was expressed by rank sum test.The measurement data consistent with normal distribution were expressed by x±s,and the t-test was used for comparison between the two groups.The risk factors were analyzed by multivariate logistic regression analysis,the significant level was 0.05,and the difference was statistically significant.ResultThe study included 477 SLE patients who met the criteria in the first affiliated Hospital of Zhengzhou University from January 2019 to June 2020,including 48 SLE patients with PAH(PAH group)and the other 429 SLE patients without PAH(without PAH group).In PAH group,pulmonary systolic pressure and tricuspid regurgitation rate were 62.92±20.73mm and 3.61±0.64m/s respectively by transthoracic echocardiography.The diameter of right ventricle,superior and inferior diameter of right atrium and left and right diameter in PAH group were 20.40±6.00mm,38.90±9.30mm,49.10±9.20mm,respectively.In the PAH group,the female patients accounted for 97.92%,the average age of onset was 41.65±11.87 years old,the average course of SLE was 36.00(4.00,105.00)months,and the SLEDAI score was 8.00(5.25,10.75).Among them,9 cases were complicated with Raynaud phenomenon,19 cases with lupus nephritis,3 cases with vasculitis,32 cases with serositis,and 14 cases with interstitial lung disease.The level of serum uric acid was 406.31±173.63 umol/L.23 cases were positive for anti-dsDNA antibodies and 33 cases were positive for anti-RNP antibodies.In non-PAH group,pulmonary systolic pressure and tricuspid regurgitation rate by transthoracic echocardiography were 24.21 ± 4.52mm and 2.22 ± 0.32m/s respectively.The diameter of right ventricle,superior and inferior diameter of right atrium and left and right diameter of right atrium in non-PAH group were 15.60±1.60mm,31.80mm±4.30mm,39.10±4.50mm respectively.In the non-PAH group,the female patients accounted for 87.88%,the average age of onset was 36.37±12.64 years old,the average course of SLE was 12.00(2.00,60.00)months,and the SLEDAI score was 7.00(4.00,10.50).Among them,97 cases were complicated with cheek erythema,26 cases with Raynaud phenomenon,182 cases with lupus nephritis,17 cases with vasculitis,111 cases with serositis and 21 cases with interstitial lung disease.The level of serum uric acid was 299.29±108.63 umol/L.Anti-dsDNA antibody was positive in 285 cases and anti-RNP antibody was positive in 221 cases.Univariate analysis of the clinical data of PAH group and non-PAH group showed that there were significant differences in female,age of onset,long course of disease,serositis,interstitial lung disease,Raynaud phenomenon,elevated CRP,elevated serum uric acid level,positive anti-RNP antibody and positive anti-dsDNA antibody between the two groups.Multivariate logistic regression analysis showed that female,long course of disease,advanced age,serositis,elevated serum uric acid level and interstitial lung disease were independent risk factors for SLE complicated with PAH.Conclusion1.In this study,we found that the first three main clinical features of SLE patients with PAH were serositis,lupus nephritis and interstitial lung disease.The first three main clinical features of SLE patients without PAH were lupus nephritis,serositis and arthritis.2.Female,advanced age,long course of disease,elevated serum uric acid level,serositis and interstitial lung disease are independent risk factors of SLE complicated with PAH,which can be used as predictive indicators of SLE complicated with PAH and provide early warning for early detection of PAH. |
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