Supplementary Table 1 Clinical characteristics of 44 patients with systemic sclerosis, grouped according to the skin involvement
From: Von Willebrand factor propeptide as a marker of disease activity in systemic sclerosis (scleroderma)
Disease form | dSSc | lSSc | suspSSc | ||||
|---|---|---|---|---|---|---|---|
No. | 13 | 23 | 8 | ||||
Sex (F/M) | 12/1 | 21/2 | 8/0 | ||||
Normal | |||||||
Median | Range (SEM) | Median | Range (SEM) | Median | Range (SEM) | values | |
Age (y) | 49 | 27–75 (4.0) | 56 | 18–76 (3.3) | 51 | 20–67 (6) | |
Duration (y) | 1 | 1–7 (0.46) | 2 | 1–49 (2.4) | – | ||
Skin score (points) | 26 | 15–36 (1.7) | 5 | 2–15 (0.81) | 3 | 2–4 (0.3) | 0 |
VC (%p) | 97 | 61–148 (6.3) | 94 | 51–120 (3.8) | 99 | 84–130 (6.0) | 80–120 |
DLCO (%p) | 77 | 46–111 (6.0) | 77 | 40–116 (4.3) | 86 | 67–105 (5.4) | 80–120 |
PAPsyst (mmHg) | 36 | 20–43 (2.9) | 30 | 18–65 (3.5) | 28 | 24–34 (1.6) | ≤ 32 |
GFR (%p) | 109 | 94–130 (4.6) | 95 | 70–136 (3.8) | 101 | 93–114 (2.6) | 80–120 |
Capillary density (loops/mm) | 5.1 | 2.9–8.3 (0.7) | 4.5 | 2.2–8.4 (0.4) | 6.9 | 5.7–7.4 (0.3) | 7.5–11.2 |
ESR (mm/h) | 18 | 2–70 (6.3) | 14 | 4–70 (3.2) | 12.5 | 2–33 (4.3) | <20 |
CRP (mg/l) | 6.5 | 5–88 (6.9) | 5 | 5–32 (1.2) | 5 | 5–26 (2.6) | <5 |
Orosomucoid (g/l) | 0.94 | 0.70–1.89 (0.11) | 0.87 | 0.42–1.63 (0.53) | 0.76 | 0.45–1.13 (0.09) | 0.55–1.05 |