Clinical Features Of Connective Tissue Disease Associated Pulmonary Artery Hypertension

OBJECTIVE: To summarize the clinical features of connective tissue disease-related pulmonary hypertension(CTD-PAH)by right heart catheterization.Baseline and follow up data were collected and investigate risk factors of death.METHOD: A total of 61 patients who were diagnosed as CTD-PAH by right ventricle catheterization from June 2008 to June 2016 in the Department of Rheumatology or Cardiology,General Hospital of Tianjin Medical University.Baseline and follow up data were collected.Cox regression was also conducted to identify the prognostic factors.To compare the clinical features of SLE-PAH(n = 30)and non-SLE-PAH(n = 31)and p SS-PAH(n = 12)and non-p SS-PAH(n = 49).RESULTS:1.61 CTD-PAH patients were enrolled,including 60 women(98.36%),male 1(9.7%),SSc(9.8%),UCTD 4 cases(6.6%),MCTD 4(9.8%),USSD(6.9%),the average age of diagnosis was 43.38 ± 14.64 years(1.6%),1 case(1.6%),1 case(1.6%),1 case(1.6%)and 1 case of APS(1.6%).The mean duration of the first PAH clinical symptoms to PAH diagnoesd was 18.93 ± 24.5 months,when PAH was diagnosed,the duration of CTD was approximately 6.71 ± 12.28 years.This Clinical manifestations of PAH sorting by frequency are: postoperative shortness of breath in 54 cases(88.5%),fatigue in 22 cases(36.1%),dry cough in 7 cases(11.5%),palpitations in 6 cases(9.8%),Chest pain in 3 cases(4.9%),syncope in 3 cases(4.9%).WHO Fc-I grade 4 cases(5.6%),WHO Fc-II grade 32 cases(52.5%),WHO Fc-III grade 22 cases(36.1%),WHO Fc-IV grade 3 cases(4.9%).there were 28 patients have renault(45.9%).The common autoantibodies is anti-SSA antibody(62.2%)、anti-n RNP antibody(39.3%)anti-SSB antibody positive(27.87%)/ anti-ds-DNA antibody(19.67%);anti-Sm antibody positive(15.51%);anti-ACA antibody positive(3.45%);anti-Scl-70 antibody positive(1.72%);ANCA is negative.The percentage of taking glucocorticoids,immunosuppressive agents and PAH-targeted drugs at baseline was 75.41%,55.74% and 75.41%,respectively.61 CTD-PAH patients were followed up for 1 to 89 months,The mean follow-up time was 31.4 ± 24.7 months,of which 7 died,2 were lost and 52 survived.The 1-year,3-year,and 5-year survival rates of CTD-PAH patients were 95%,90% and 83%.RVEDP was an independent risk factor of death in CTD-PAH patients.2.SLE-PAH is younger than non-SLE-PAH in baseline age(P = 0.004).The level of Immunoglobulin is higher is higher in SLE-PAH than non-SLE-PAH group(P = 0.049).The SLE-PAH group showed more urinary protein and urinary blood than non-SLE-PAH group(P= 0.002 and < 0.001 respectively).The m RAP of SLE-PAH and non-SLE-PAH a 7.33 ± 4.32 mm Hg and 4.87 ± 2.47 mm Hg,The SLE-PAH is higher(P = 0.01).The RVEDP of SLE-PAH and non-SLE-PAH group are 10.93 ± 4.32 mm Hg and 8.43 ± 2.91 mm Hg,the fomer is higher(P = 0.01).The the proportion of SLE-PAH treated with glucocorticoid and PAH targeting at baseline is higher than non-SLE-PAH drugs(P = 0.002).The survival rates of SLE-PAH and non-SLE-PAH is not different.3.There were 12 patients with p SS-PAH,all female,average age is 53.92 ± 15.3 years old,49 patients with non-p SS-PAH,1 male and 48 female,average age is 40.80 ± 13.41 years old,non-p SS-PAH was older(P = 0.02).There were 2 cases of p SS-PAH has Renault,26 non-p SS-PAH patients have Renault(P = 0.03).The syncope is more common in p SS-PAH group(P = 0.006).The positive rate of anti-SSA antibody and anti-SSB antibody are more higher,and the positive rate of anti-n RNP antibody is lower in p SS-PAH than non-p SS-PAH(P = 0.02,0.002 and 0.002 respectively).There was no significant difference in hemodynamic parameters between p SS-PAH and non-p SS-PAH baseline.Conclusion 1.The SLE-PAH is more common in CTD-PAH,followed by p SS-PAH.2.RVEDP is an independent risk factor of death in CTD-PAH.3.The baseline hemodynamics is much worse in SLE-PAH than non-SLE-PAH.More SLE-PAH patients take glucocorticoid and PAH targeted therapy.4.p SS-PAH patients often appear syncope,anti-SSA antibodies may be related to the pathogenes of p SS-PAH.