Clinical Characteristics And Survival Of Connective Tissue Diseases With Pulmonaryarterial Hypertension

Objective This study was aimed to investigate the clinical characteristics and survival of three type of connective tissue disease associated pulmonary arterial hypertension(CTD-PAH),to provide a comprehensive understanding of CTD-PAH.Methods The study was retrospectively collected 218 PAH patients with CTD-PAH,included 120 with systemic lupus erythematosus(SLE),64 with systemic sclerosis(SSc),and 34 with primary Sj?gren’s syndrome(pSS)who was hospitalized in our hospital between October 2012 and January 2018.PAH was diagnosed based on pulmonary arterial systolic pressure(PASP)≥35 mmHg as measured by Doppler echocardiography and was divided into mild(35-50mmHg),moderate(51-70mmHg),and severe PAH(≥71mmHg)according to the degree of PASP.Baseline demographics,clinical features,laboratory results,and echocardiography assessments were analyzed.Kaplan–Meier method was used to calculate the survival rates.Cox proportional hazards regression analysis was used to identify independent factors of mortality.Results:1.The SSc patients were more likely to have PAH [64/497(12.9%)] as theunderlying CTDs were pSS [34/523(6.5%)] and SLE [120/3133(3.8%)].2.The Mean age of CTD-PAH was 47.47 ± 38.70 years at the time of PAH diagnosis,and patients with SLE were younger(47.47±38.70 years old)than SSc(58.02±10.93 years old)and pSS(52.09±16.08 years old).3.The most common clinical symptoms of CTD-PAH was dyspnea(65.6%),and followed by cough(51.4%),expectoration(39.9%),Renault phenomenon(38.5%),edema(34.4%),chest tightness(25.7%),and chest pain(14.2%).WHO-FC III/IV were found in 42.2% patients of CTD-PAH,which was 53.2% for SSc,44.2% for SLE,and 35.3% for pSS.The lung involvement in CTD-PAH from high to low were SSc(81.2%),pSS(58.8%)and SLE(30.0%),respectively,and the differences were statistically significant.The pulmonary arterial pressure of CTD-PAH was mainly mildly to moderately elevated(80.3%),and PASP in pSS group was higher than that in SLE and SSc group(p<0.05).4.Symptoms of dyspnea,expectoration and chest tightness were more common in the non-survivors than in the survivors.The proportion of WHO-FC III/IV patients in the non-survivors was 58.9%,which was statistically higher than the survivors(35%)(p=0.003).Compared with the survivors,the non-survivors of CTD-PAH showed higher PASP(51.85±18.61 vs63.46±23.02mmHg),with moderately and severely elevating in pulmonary artery pressure(62.5% vs 36.8%,p=0.001),and higher incidence of right atrium enlargement and right ventricle enlargement(p<0.05).In the SSc-PAH and pSS-PAH groups,the HRCT score of Interstitial lung disease(ILD)was higher in the non-survivors than that in the survivors(p<0.05).The survivors of SLE-PAH were with higher positive of homogeneous type of Antinuclear antibodies(ANA).Higher level of Immunoglobulin G(IgG)and the EuropeanUnion for resistance rheumatism SS disease activity index score(ESSDAI)was showed in pSS-PAH survivors.5.All patients were treated with glucocorticoids and immunosuppressive agents.94(43.1%)patients were received the treatment of PAH targeted drugs,and 19(20.4%)patients received combination therapy,but there was no statistical difference between the non-survivors and survivors.The outcomes of173 patients were eventually followed up,including 98 patients with SLE,47 with SSc and 28 with pSS,and the mortality rates was 26.3%,48.8%,41.7%,respectively.The overall survival rate of CTD-PAH was 81.4%,72.4% and56.9% at 1,3 and 5 years,among that SSc had the lowest survival rate,but there was no statistical difference among the three subgroups of CTD-PAH.There was no statistically significant difference in mortality between the limited and diffuse SSc-PAH [43.8%(7/16)vs.45.2%(14/31),p=0.927].6.In CTD-PAH patients,cox regression univariate analysis showed that age≥50 years old,the severity of PAH,the enlargement of right ventricular,WHO-FC III/IV,and the symptom of dyspnea and expectoration were risk factors of death.However,results of multivariate analysis confirmed that age over 50 years old was the only independent risk factor of mortality.Conclusions:1.The most common clinical symptoms of CTD-PAH were dyspnea and cough,and 42.2% of patients were admitted to the WHO-FC III/IV.SLE-PAH patients have the youngest onset age.The prevalence of SSc-PAH was the highest,and with the most common pulmonary involvement,the lowest survival rate and the worst prognosis.PASP in pSS patients was higher than that in SLE and SSc patients.2.Overall survival rates of CTD-PAH at 1,3 and 5 years were 81.4%,72.4% and 56.9%,respectively.The mortality rates of SLE-PAH,SSc-PAH and pSS-PAH were 26.3%,48.8% and 41.7%,respectively.3.Age,dyspnea,severity of PAH,right ventricular enlargement,and WHO-FC III/IV might predict the poorer prognosis of CTD-PAH,and age over50 years was as the independent predictor of mortality.