Clinical Characteristics And Therapeutic Effect Of Systemic Sclerosis Combined With Pulmonary Hypertension

Objective To analyze the clinical features and therapeutic effects of patients with systemic sclerosis combined with pulmonary hypertension(SSc-PAH).Methods We retrospectivly analyzed the clinical material of 103 patients with SSc who were hospitalized from Department of Rheumatology,the First Affiliated Hospital of Guangxi Medical University from October 2002 to November 2016.All patients were divided into SSc-nonPAH group and SSc-PAH group.The SSc-PAH group was divided into mild(PASP:35-50mmHg),moderate(PASP:51-70 mmHg)and severe(PASP:> 70 mmHg)according to the degree of systolic blood pressure of the pulmonary artery based on cardiac color Doppler ultrasound.A comparison between each group would be done,including the general data,clinical manifestations,laboratory findings,treatment and outcome.Results1.103 cases of SSc patients,including 42 males and 61 females,aging from15 to 81,The average age is 52.59 ± 12.55 years.The duration was from 1mouths to 32.75 years,and the median duration was 39 mouths.Among them,53 cases of SSc-PAH patients,mild in 17 cases,moderate in 28 cases,severe in 8cases.SSc can accumulate the body of the system,the most common clinical manifestations of the Renault phenomenon(90.3%)and fingertips skin hardening(100%).2.The median duration of SSc-PAH was 52 months,which was significantly longer than that in SSc-nonPAH group(36 months).The incidence of gastrointestinal involvement(24.53%VS.8%),postoperative shortness of breath(64.15%VS.44%),pericardial effusion(41.51%VS.14%)was significantly higher in SSc-PAH group than in SSc-nonPAH group.While the incidence of nucleolus(52.83%VS.76%)and anti-Scl-70 antibody(69.81%VS.89.8%)in SSc-PAH group was lower than that in SSc-nonPAH group.The difference was statistically significant(P <0.05).There were no significant differences in age and sex between the two groups.3.In the SSc-PAH group,the left ventricular diastolic function decreased,pericardial effusion,right atrioventricular enlargement,the main pulmonary artery diameter widening was significantly higher than the SSc-nonPAH group(P <0.05).The patients with SSc-PAH combined with pulmonary interstitial lesions were more severe than SSc-nonPAH group.4.The scores of chest HRCT score(12,20)VS.(9,12),P <0.05 in SSc-PAH group were higher than those in SSc-nonPAH group.5.53 patients with SSc-PAH were divided into mild,moderate and severe groups.The median and moderate course of mild to moderate group(52VS.34.5VS.94 months,P <0.05)were significantly shorter in the severe group.The incidence of muscle involvement in moderate and moderate groups was higher than that in mild and severe groups(60.71% vs.23.53%,0%,P<0.01).(CRP),creatine kinase(CK)levels(17.15 VS.11.6,5.26 mg / L,185 VS.95,62.25 U / L,P(P <0.05),and the levels of C-reactive protein(CRP)(P <0.05).The hemoglobin(HCG)level was lower than that of the other two groups(P<0.05).The levels of complement C3(Complement 3,C3)in the severe group were significantly higher than those in the mild and moderate groups(1.07 VS.0.88,0.76 g / L,P <0.05),but the positive rate of anti-Scl-70 antibody(12.5% vs 82.35%,78.57%,P <0.05)were lower than those in the first two groups.6.In the comparison of echocardiography,the left ventricular diastolic function decreased(97.5% vs.71.43%,29.41%),the right atrium was increased(62.5% vs.14.29%,5.88%),the main pulmonary artery diameter widened 100%vs.25.57%,0%)were significantly higher than those in mild and moderate groups(P <0.05).There was no significant difference in HRCT scores between the three groups.7.Binary logistic regression analysis showed that the nucleolus type was a protective factor for SSc-PAH,and pericardial effusion was an independent risk factor.8.53 patients with PAH were treated with prednisone(5-40 mg / d)and immunosuppressive agents(cyclophosphamide(42 cases),azathioprine(4 cases),cyclosporine(3 cases),methylamine(5 cases),leflunomide(3 cases),thalidomide(6 cases)and other drugs;treatment of pulmonary artery pressure(Pulmonary artery pressure,PAP)all with sodium vertein sodium,moderate to severe group Patients with sildenafil / tadalafil in 12 cases,plus bosentan 6 cases.There were 23 patients who underwent cardiopulmonary bypass after 6 months of treatment.Among them,11 patients were treated with reduced PAP therapy,7cases had decreased PASP,4 cases had no descent or aggravation in PASP,12 cases had no use of PAP drugs,PASP decreased,10 cases did not decline oraggravated PASP(P <0.05).Conclusion1.SSc-PAH is more common in patients with mild to moderate PASP;longer duration than SSc-nonPAH patients.2.PAH is a common complication of SSc.Compared with SSc-nonPAH patients,the pulmonary interstitial lesions(ILD)of SSc-PAH patients are heavier,and the incidence of puberty and pericardial effusion is more common in digestive tract involvement,ANA nucleolus type,anti-Scl-70 antibody positive rate was significantly lower.3.Compared with patients with severe SSc-PAH,mild and moderate groups of patients with shorter course of disease,anti-Scl-70 antibody positive rate is low,but C3 levels.Moderate SSc-PAH patients are more likely to have muscle involvement,elevated CRP and CK levels,and decreased HGB levels.4.The risk factor for SSc-PAH is pericardial effusion,whereas ANA nucleolus is a protective factor5.SSc-PAH patients in addition to the use of hormones and immunosuppressive therapy,plus lower pulmonary artery pressure targeted drug therapy have a better effect.