Patient characteristics with frequencies of sex, SSc subsets, ANA, organ involvement, current symptoms, and vasoactive therapies together with (A) the frequency of missing data for all patients and for 2 different age groups, including (B) the frequencies as well as the p values (Fisher’s exact test).
| Characteristics | (A) | (B) | |||
|---|---|---|---|---|---|
| Total, % (n) | Missing Data, % (n) | Age 18–65 Yrs, n = 2439 | Age > 65 Yrs, n = 697 | p | |
| Female | 81.7 (2652/3248) | 0.2 (5/3248) | 80.9 | 83.9 | 0.076 |
| Male | 18.2 (591/3248) | 19.1 | 16.1 | ||
| lcSSc | 47.9 (1555/3248) | 2.0 (64/3248) | 52.5 | 62.5 | < 0.0001 |
| dcSSc | 30.0 (976/3248) | 36.1 | 27.4 | ||
| SSc overlap syndrome | 10.0 (325/3248) | 11.4 | 10.1 | ||
| ANA | |||||
| Scl-70/ATA-positive | 26.4 (859/3248) | 7.0 (226/3248) | 30.4 | 21.6 | < 0.0001 |
| ACA-positive | 34.3 (1113/3248) | 6.2 (202/3248) | 34.5 | 46.0 | < 0.0001 |
| Organ involvement | |||||
| RP | 93.2 (3026/3248) | 1.9 (62/3248) | 95.7 | 93.6 | 0.031 |
| PAH | 13.4 (434/3248) | 8.5 (275/3248) | 13.4 | 20.3 | < 0.0001 |
| Kidney | 8.7 (281/3248) | 8.5 (275/3248) | 9.5 | 10.0 | 0.695 |
| Heart | 12.6 (409/3248) | 8.4 (273/3248) | 13.6 | 14.2 | 0.688 |
| Current symptoms | |||||
| DU | 25.7 (836/3248) | 4.3 (139/3248) | 28.5 | 20.5 | < 0.0001 |
| HTN | 22.7 (736/3248) | 4.8 (156/3248) | 22.2 | 30.5 | < 0.0001 |
| Renal insufficiency | 10.7 (347/3248) | 5.3 (173/3248) | 10.8 | 13.5 | 0.060 |
| Proteinuria | 8.1 (263/3248) | 6.1 (199/3248) | 8.9 | 7.6 | 0.304 |
| Vasoactive therapies | |||||
| Vasoactive drugs | 61.1 (1984/3248) | 4.4 (144/3248) | 63.9 | 65.7 | 0.409 |
| ACE inhibitors | 34.2 (678/1984) | 0.3 (6/1984) | 33.7 | 37.0 | 0.228 |
| CCI | 47.6 (945/1984) | 10.1 (200/1984) | 54.2 | 49.1 | 0.084 |
| PDE5 inhibitors | 4.1 (82/1984) | 10.1 (200/1984) | 4.6 | 4.4 | 1.000 |
| ET1RA | 8.7 (173/1984) | 10.1 (200/1984) | 9.4 | 10.5 | 0.497 |
| ß-blocker | 10.6 (211/1984) | 10.1 (200/1984) | 11.0 | 15.2 | 0.033 |
| α-blocker | 1.0 (19/1984) | 10.1 (200/1984) | 0.7 | 2.3 | 0.013 |
| AT1RA | 8.8 (175/1984) | 10.1 (200/1984) | 8.4 | 14.9 | < 0.0001 |
| Pentoxifylline | 10.1 (201/1984) | 10.1 (200/1984) | 12.4 | 8.2 | 0.030 |
| IV prostanoids | 21.1 (419/1984) | 10.1 (200/1984) | 24.9 | 19.5 | 0.030 |
| Topical vasoactive treatment | 2.4 (48/1984) | 10.1 (200/1984) | 2.6 | 3.1 | 0.725 |
SSc: systemic sclerosis; ANA: antinuclear antibodies; lcSSc: limited cutaneous SSc; dcSSc: diffuse cutaneous SSc; ATA: antitopoisomerase antibody; ACA: anticentromere antibody; RP: Raynaud phenomenon; PAH: pulmonary arterial hypertension; DU: digital ulcers; HTN: hypertension; ACE: angiotensin-converting enzyme; CCI: calcium channel inhibitors; PDE5: phosphodiesterase type 5; ET1RA: endothelin 1 receptor antagonists; AT1RA: angiotensin 1 receptor antagonists; IV: intravenous.