No. Patients | Total Cohort, n = 279 | RNAP, n = 33 (12%) | ACA, n = 134 (48%) | ATA, n = 46 (16%) | Other Ab, n = 55 (20%) | No Ab, n = 11 (4%) | p* | p** |
---|---|---|---|---|---|---|---|---|
Time from onset to PH, yrs, mean (SD) | 8.5 (7.9) | 7.6 (10.0) | 8.3 (9.2) | 11.8 (7.6) | 7.4 (5.0) | 8.3 (3.9) | 0.413 | 0.886 |
PH | 51 (18) | 4 (12) | 28 (21) | 5 (11) | 11 (20) | 3 (27.3) | 0.796 | 0.105 |
PAH | 33 (12) | 1 (3) | 24 (18) | 1 (2) | 6 (11) | 1 (9) | 0.722 | 0.025 |
PH-ILD | 18 (6) | 3 (9) | 4 (3) | 4 (9) | 5 (9) | 2 (18) | 0.858 | 0.025 |
mPAP at PH diagnosis, mmHg, mean (SD) | 29.9 (11.9) | 25.3 (10.1) | 30.7 (12.4) | 26.0 (7.5) | 32.8 (12.7) | 39.8 (10.8) | 0.485 | 0.064 |
Baseline sPAP, mmHg, mean (SD) | 27.7 (19.8) | 25 (14.8) | 28.8 (20.1) | 24.0 (9.3) | 28.5 (25.8) | 37.2 (19.4) | 0.725 | 0.333 |
Followup sPAP, mmHg, mean (SD) | 34.9 (23.7) | 30.4 (17.7) | 32.1 (16.4) | 36.6 (26.4) | 34.5 (24) | 46.4 (31.1) | 0.564 | 0.684 |
Baseline NT-ProBNP, pmol/l, mean (SD) | 78.2 (321.5) | 25.7 (80.1) | 59.9 (198.9) | 31.6 (50.1) | 58.9 (134.8) | 53.7 (49.2) | 0.134 | 0.191 |
Followup NT-ProBNP, pmol/l, mean (SD) | 218.7 (606.8) | 287.6 (780.1) | 221.7 (701.0) | 106.9 (606.1) | 189.4 (412.9) | 298.4 (485.9) | 0.807 | 0.713 |
Myocardial infarction | 24 (9) | 1 (2) | 12 (9) | 3 (9) | 5 (9) | 3 (27) | 0.222 | 0.636 |
Angina pectoris | 23 (8) | 0 (0) | 14 (11) | 3 (7) | 5 (9) | 1 (9) | 0.183 | 0.038 |
↵* p values between RNAP and ATA.
↵** p values between RNAP and ACA. PH: pulmonary hypertension; NT-proBNP: N-terminal pro-brain natriuretic peptide; sPAP: systolic pulmonary artery pressure on echocardiogram; RNAP: RNA polymerase III; ACA: anticentromere antibody; ATA: antitopoisomerase I; Ab: antibody; PAH: pulmonary arterial hypertension; ILD: interstitial lung disease; mPAP: mean pulmonary artery pressure by right heart catheterization, data shown from PH patients.