| Objectives:We establish primary Sjogren syndrome (pSS) associated pulmonary arterial hypertension (PAH) and systemic sclerosis (SSc) associated PAH cohort to provide baseline characteristics of right heart catheterization (RHC) and outcomes based on Peking Union Medical College Hospital (PUMCH) rheumatology clinic. Derive risk factors of PAH onset among pSS-PAH cases. Investigate prognostic factors associated with estimated survival and treat-to target rate. Examine whether genetic polymorphisms carry susceptibility for PAH in systemic lupus erythematosus (SLE) cases.Methods:We establish RHC based pSS-PAH and SSc-PAH cohort by registering patients of PUMCH rheumatology clinic. Clinical records of RHC baseline and follow-up visits are collected. Characteristics of RHC baseline are analysed. To derive risk factors of PAH onset among pSS populations, we compare pSS-non-PAH with pSS-PAH cohort by applying Logistic regression. Estimated survival and treat-to target rate based on time of diagnosis is established. Then we use Cox analysis to investigate independent prognostic factors of outcomes. By determining SNPs among SLE-PAH, SLE-non-PAH and health controls respectively, we examine associations between genetic background with PAH onset.Results:1.29 cases are classified as pSS-PAH. Age of RHC diagnosis is 40.62±8.99 years. PAH disease duration when RHC diagnosis is 24.66±31.88 months. Anti-RNP antibody are positive in 6 (20.69%) case. Mean pulmonary arterial pressure (mPAP) is 51.79±9.98mmHg, pulmonary arterial wedge pressure (PAWP) is 8.72±3.12mmHg, pulmonary vascular resistance (PVR) is 13.01±5.95WU, cardiac index is 2.29±0.78L/(min*m2), right atrial pressure is 7.57±4.78mmHg.2 cases (7.41%) respond to acute vasodilator test. Echocardiography indicate right ventricular antero-posterior diameter as 32.5±7.47mm, tricuspid anular plane systolic excursion as 16.2±2.95mm and pericardial effusion in 9 cases (31.03%).14 cases (51.85%) are graded with NYHA functional class II,13 cases as class III. 6-minute walk distance is 406±85.15m. BNP level is 462.95±468.94ng/L. DLCO is 62.28±8.83% predicted.25 cases (86.21%) receive immunosuppressive therapy at baseline.25 cases receive PAH therapy.24 cases (82.76%) are treated with combination therapy overall. Risk factors for PAH in pSS patients:ocular symptoms (p=0.000,OR=0.044,95% CI=0.009-0.218), fever (p=0.012,OR=0.053,95% CI=0.005-0.523), Raynaud’s phenomenon (p=0.001,OR=15.109,95% CI=2.929-77.936) and renal involvement (p=0.001, HR=0.034,95% CI=0.005-0.240).1,3 and 5-year estimated survival rates are 80.2%,74.8% and 67.4% respectively.1,3 and 5-year treat-to-target rates are 28.6%,43.8% and 62.5% respectively. Multivariate Cox analysis reveals no independent prognostic factors.2. 50 cases are included in SSc-PAH cohort. Age of RHC diagnosis is 43.26±12.02 years. PAH disease duration when RHC diagnosis is 24.62±32.65 months. Anti-centromere antibody is tested positive in 12 cases (25.53%). Anti-RNP antibody is positive in 29 cases (60.41%). No positive anti-Scl-70 antibody is reported. mPAP is 42.88±10.84mmHg, PAWP is 10.33±1.51mmHg, PVR is 8.78±3.24WU, cardiac index is 2.68±0.75L/(min*mz), right atrial pressure is 5.50±3.90mmHg.3 cases (6.82%) respond to acute vasodilator test. Echocardiography indicate right ventricular antero-posterior diameter as 29.84±5.61mm, tricuspid anular plane systolic excursion as 16.71±4.10mm and pericardial effusion in 17 cases (34%).17 cases (42.5%) are graded with NYHA functional class Ⅱ,21 cases (52.5%) as class Ⅲ,2 other cases (5%) as class IV.6-minute walk distance is 388.45±95.41m. BNP level is 366.74±503.94ng/L. DLCO is 45.75±16.92% predicted.45 cases (90%) receive immunosuppressive therapy at baseline.40 cases (80%) receive PAH therapy.40 cases (80%) are treated with combination therapy overall.1,3 and 5-year estimated survival rates are 72.5%,58.8% and 43.9% respectively. Multivariate Cox analysis reveals left ventricular ejection fraction (p=0.036, HR=0.865, 95% CI=0.756-0.990) as an independent protector for death, while right ventricular antero-posterior diameter (p=0.027, HR=1.172,95% CI=1.019-1.347) as an independent predictor for death.1,3 and 5-year treat-to-target rates are 20.7.6%,45.2% and 45.2% respectively. Multivariate Cox analysis reveals baseline PAH disease duration (p=0.01, HR=1.039,95% CI=1.016-1.063) and IgG level (p=0.028, HR=1.115,95% CI=1.012-1.229) as independent predictors for treat-to-target, while RDW-C (p=0.036, HR=0.396,95% CI=0.167-0.939) as an independent risk factor.3.88 SLE-PAH cases,156 SLE-non-PAH cases and 142 health controls are included in SNP association study. A total of 28 SNP locus are tested. G-allele of CBLN2, rs2217560 locus is found associated with PAH onset in SLE population of our center (p=0.018,OR=1.95,95% CI=1.116-3.412).Conclusions:We established the largest ever pSS-PAH cohort and extended patients enrollment to SSc-PAH cohort. Baseline characteristics, risk factors for PAH onset and outcomes are reported. Prognostic factors are determined. We discovered SNP locus associated with PAH onset in SLE population of our center.
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