Risk Factors And Prognostic Analysis Of Pulmonary Arterial Hypertension-connective Tissue Disease

Background:Connective tissue disease(CTD)is a kind of non-infectious chronic systemic autoimmune disease,including systemic lupus erythematosus(SLE).The main feature of CTD is systemic organ injury caused by systemic vascular and connective tissue involvement.The lung is one of the most frequently damaged organs by CTD because of its abundant connective tissue and blood vessels.The main manifestations of CTD involving interstitial lung disease(ILD),pulmonary arterial hypertension(PAH),pulmonary thromboembolism,pulmonary alveolar hemorrhage,pleurisy and infection.Connective tissue disease-Pulmonary arterial hypertension(CTD-PAH)is one of the important causes of death in patients with CTD.Objective: To compare the clinical indexes of patients with CTD-PAH and CTD without PAH(CTD-NPAH).Tto analyze the risk factors and prognosis of CTD-PAH.Analyze and compare the differences of clinical features between CTD-PAH and PAH patients with unknown causes,so as to provide basis for the diagnosis,treatment and improvement of prognosis of clinical CTD-PAH.Methods:Randomly select 43 patients with CTD-PAH,50 patients with CTD-NPAH and 50 patients with unexplained PAH who were treated at Dalian Central Hospital from January 2010 to December 2020.By comparing sex,age,course of disease,complication of other systemic diseases,rash,joint involvement,Raynaud’s phenomenon,cough,shortness of breath,cardiac function classification.N-terminal pro-brain natriuretic peptide(NT-pro-BNP),partial ions,liver and kidney function,blood lipids,blood routine,thyroid function,inflammatory indexes,autoantibodies,immunoglobulins,complements,lymphocyte subsets,arterial blood gas analysis,electrocardiogram,echocardiography,pulmonary function,medication,prognosis and other indicators to analyze the risk factors of inducing CTD-PAH,the clinical characteristics of CTD-PAH patients and the main factors affecting its prognosis.The statistical analysis of the data was performed by SPSS20.0 software,and the test results were statistically significant with P<0.05.Results:Compared with the CTD-NPAH group,patients with CTD-PAH have a longer course of CTD.In the CTD period without PAH,they have more Raynaud’s phenomenon,cough,shortness of breath,right-sided ECG,pericardial effusion,etc.They are more likely to be accompanied by heart disease,kidney disease and other lung diseases,worse grades of heart function,higher mortality,absolute count of neutrophils(NEUT),neutrophil lymphocyte ratio(NLR),lactate dehydrogenase(LDH),uric acid(UA),D-Dimer(D-D),NT-pro-BNP,erythrocyte sedimentation rate(ESR),C-reactive protein(CRP)and right ventricular diameter(RVD)levels,lower percentage of lymphocytes(LY%),T-helper lymphocyte Absolute count(Th count)levels.Among them,in the CTD period without PAH,accompanied by heart disease [HR=3.967,95%CI(1.091,14.422),P=0.036],high DD [HR=1.509,95%CI(1.091,14.422),P=0.036],high LDH [HR=1.001,95%CI(1.000,1.001),P=0.021],high NLR [HR=1.116,95% CI(1.116,1.009),P=0.032] is independent risk factors for CTD complicated by PAH..Compared with SLE-NPAH patients,patients with SLE-PAH have a longer course of CTD.During the period of SLE without PAH,their NEUT and NLR levels are higher,the positive rate of anti-double-strand-DNA antibodies is lower,and the percentage of percentage of T-suppressor lymphocyte(Ts%),LY%,and left ventricular diameter(LVD)values are lower.Among them,in the SLE period without PAH,high NLR [HR=1.377,95%CI(1.138,1.667),P=0.001] is an independent risk factor for SLE complicated by PAH.Compared with the unexplained PAH group,patients with CTD-PAH during PAH have more women with more disease,younger age of onset and diagnosis of PAH,shorter course of PAH,and more likely to have other lung diseases,kidney diseases and heart diseases,but heart function Lower grade,higher levels of anti-thyroglobulin antibodies(TGAb)and maximum tricuspid regurgitation velocity(TRVmax),low levels of erleft atrial diameter(LAD),right atrial diameter(RAD)from left to right,RAD-anteroposterior diameter and Carbon Monoxide Diffusing Capacity(DLCO).Among them,during the PAH period RAD [OR=1.203,95%CI(1.050,1.379),P=0.008],DLCO [OR=1.225,95%CI(1.105,1.359),P<0.001],whether with heart Disease [OR=6.022,95%CI(1.310,27.687),P=0.021],whether it is accompanied by kidney disease [OR=16.268,95%CI(2.295,115.322),P=0.005] are independent and significant differences between the two groups.In the CTD-PAH group,during PAH,patients in the CTD-PAH group were diagnosed with LVD,RAD from left to right and cough had independent linear correlations with pulmonary artery systolic pressure(PASP).The regression equation is PASP=26.917-0.976*(LVD)+1.443*(RAD-left and right diameter)+15.151*(cough)(1with 0 without).After testing this equation,73.9% of the PASP results can be analyzed.During PAH,the results of survival analysis in the CTD-PAH group showed high NT-pro-BNP concentration [HR=1.000073,95%CI(1.000021,1.0001125),P=0.006],anti Scl-70 antibody positive [HR=7.756,95% CI(2.088,28.883),P=002],low DLCO[HR=0.909,95% CI(0.845,0.978),P=0.11] are independent risk factors for poor prognosis of CTD-PAH.Conclusion:1.The independent risk factors of PAH in patients with CTD are high NLR,high D-D,high LDH and heart disease,and the independent risk factors of PAH in patients with SLE are high NLR.2.Compared with other PAH patients,CTD-PAH patients have higher RAD from left to right and DLCO levels,and are more likely to be associated with heart disease and kidney disease during PAH.3.The PASP of CTD-PAH patients was significantly correlated with low LVD,high RAD from left to right and cough symptoms.4.The main factors affecting the prognosis of patients with CTD-PAH were positive anti Scl-70 antibody,high NT-pro-BNP and high DLCO.