A Clinical Study Of Arteritis Involving The Pulmonary Artery

Part ?:Clinical characteristics analysis of Takayasu arteritis with pulmonary artery involvementObjective:We aim to analyze the clinical characteristics of Takaysua arteritis with pulmonary artery involvement,and to improve physicians' understandings of the disease.Methods:Patients who were diagnosed as Takaysua arteritis with pulmonary artery involvement by pulmonary angiogram or contrast-enhanced computed tomographic pulmonary and/or aorta angiography(CTA)were enrolled between January 2006 and April 2017.Comprehensive clinical data including chief complaints,World Health Organization(WHO)function class,electrocardiogram examination,six minute walking distance test and echocardiogram were evaluated.The results of laboratory test,imaging records of pulmonary angiogram/CTPA and pulmonary perfusion/ventilation were also collected.Results:101 patients of Takaysua arteritis with pulmonary artery involvement were enrolled in this study.The male to female ratio was 1:3.04.The first symptom occurred at a mean age of 34.38±9.67 years old,patients endured a mean period of 6.05±6.28 years to gain a final diagnosis.Chief complaints included shortness of breath,decreased exercise tolerance,hemoptysis and chest pain.56.6%patients were in WHO III when admission.Half patients had right ventricle overloading signs such as right ventricle hypertrophy and V1-3 T wave inversionon on ECG.Echocardiograms demonstrated enlarged right ventricle and decreased tricuspid annular plane systolic excursion.The estimated systolic pulmonary arterial pressure was 90.44±22.90mmHg,and pericardium effusion was presented in 11.9%patients.39.7%-40%patients showed elevated Creactive protein and erythrocyte sedimentation rate,and the mean N-terminal pro-brain natriuretic peptide was 1643.08±1640.20pg/ml.All patients showed pulmonary artery involvements on imaging examination,32.7%patients also with aorta and its mainbranches involved.The specific CTA manifestations included restriction,occlusion and dilatation.Both sides of pulmonary arteries were affected in 89%patients,with each segment might be affected;isolated pulmonary artery was affected more often in the right side than the left.88.2%patients had a wider main pulmonary trunk than their ascending aorta neighbor.V/Q scan showed diffused pulmonary segmental andsub-segmental perfusion defects.5-10 segments were defected in 11.9%patients while 52.5%patients showed more than 10 pulmonary segments affected.Patients with both aorta and pulmonary artery affected were comparable with isolated pulmonary artery involvement in basic clinical characteristics,laboratory results and echocardiographic findings.Conclusions:Young females were more easily suffered from Takaysua arteritis with pulmonary artery involvement.Common symptoms included shortness of breath,decreasing exercise capacity,chest pain and hemoptysis.ECG and echocardiogram showed right ventricle overloaded.Typical imaging findings included main pulmonary trunk dilation,diffused pulmonary segment and sub-segment restriction/occlusion,with some dilation presented.Part ?:Hemodynamic characteristics of pulmonary arteritis with pulmonary artery involvementObjective:We aimed to analyze hemodynamic characteristics of Takayasu arteritis with pulmonary artery involvement(pulmonary arteritis for short,PA)and compared it with chronic thromboembolic pulmonary hypertension(CTEPH)and idiopathic pulmonary arterial hypertension(IPAH).Methods:This retrospective study included PA,CTEPH and IP AH patients whom were diagnosed in Fuwai hospital between January 2006 and April 2017.Hemodynamic parameters were analyzed.Results:Forty PA patients,with mean age of 39.53±10.6 years old and average disease duration of 5.73±5.21 years were enrolled in the study.All PA patients showed elevated systolic pulmonary artery pressure of 89.03±25.39mmHg,enlarged end diastolic right ventricular diameter(31.19±5.81mm)and decreased tricuspic annular plane systolic excursion(15.89±3.89mm)on echocardiogram.Right heart catheterization showed elevated mean pulmonary artery pressure and pulmonary vascular resistance(PVR)(48.8±15.51mmHg and 11.63±6.02Wood Unit,respectively).Thirty-four CTEPH and 75 IP AH patients were also enrolled in the study.Echocardiogram showed moderate to severe pulmonary hypertension,right ventricles enlargement with decreased right heart function in all three groups.IP AH patients demonstrated longer 6MWD(433.43± 101.26 vs.367.44±72.79 and 404.68±89.28 meters,respectively,p=0.033)than PA and CTEPH patients.Pulmonary arterial pulse pressure(PP)was 67.3±22.91mmHg in PA group,which was significantly higher than CTEPH(57.88±12.68mmHg)and IP AH(48.33±15.13mmHg),p=0.000.Other hemodynamic parameters including cardiac index(CI),PVR and pulmonary vascular compliance(Cp)were comparable.Multiple linear regression analyses demonstrated PVR was negatively associated with Cp and CI,and positively associated with PP.On the other hand,Cp was positively related with CI.All three group patients shown significant negative relevance between PVR and Cp.Conclusions:PA patients' echocardiograms shown moderate to severe pulmonary hypertension and impaired right heart function.PA subjects demonstrated significantly larger pulmonary pulse pressure difference than CTEPH and IP AH,indicating more severe vascular stiffness.Part ?:Analysis of prognostic indicators of Takayasu arteritis with pulmonary artery involvementObjectives:The study aimed to investigate prognostic factors of Takayasu arteritis with pulmonary artery involvement(pulmonary arteritis,PA)and to improve fundamental understandings of the disease.Methods:Clinical charts of PA patients of PA by pulmonary artery angiography or computed tomography(CTA)in Fuwai hospital between January 2006 and April 2017 were retrospectively collected and analyzed.Comprehensive clinical records including WHO function class,echocardiography,hemodynamic values of right heart catheterization(RHC),results of laboratory test and imaging data were collected.Results:87 PA patients were enrolled in the study.PA is predominant in young female;the average disease duration was 5.44±6.10 years,with WHO classification mainly in II and III.Aorta and its main branches were also affected in 34.5%patients.Pulmonary arteries showed various extents of stenosis,occlusion or dilatation,and average 12 pulmonary segments were affected.The mean pulmonary artery pressure on RHC was 46.97±13.49mmHg and pulmonary vascular resistance was 11.39±5.76Wood Unit,with reduced cardiac index(2.53±0.80 L/min*m2).The average follow-up period was 38.49±31.16 months.Fourteen patients(16.1%)deceased at the end of follow-up,and half of the patients became better or remained stable.The deceased subjects were older and with higher WHO classification.NT-proBNP was significantly elevated while erythrocyte sedimentation rate was normal.Uni-variable Cox regression analysis showed higher WHO classification,older than 51 years and NT-proBNP higher than 3500pg/ml were negatively associated with prognosis.Multi-variable Cox regression analysis showed NT-proBNP higher than 3500pg/ml was independent poor survival predictor,with HR of 5.246(p=0.019).Conclusion:Pulmonary hypertension and right heart dysfunction are considered severe complications of Takayasu arteritis with pulmonary artery involvement.Higher WHO functional classification on admission,older age and higher level were associated with poor prognosis.Multi-variable Cox regression analysis showed NT-proBNP higher than 3500pg/ml was independent poor survival predictor.