Subset | Sex | NGF | vWF | ACE | DTPA | Tlco | Skin score | Heart invol. | Joint invol. | PNS invol. | HRCT | |
1 | L | F | 17.5 | 1340 | 1.3 | 40 | 106 | 17 | No | No | Yes | 1 |
2 | L | F | 27 | 1240 | 5.2 | 52 | 80 | 8 | Yes | No | Yes | 1 |
3 | L | M | 51 | 1410 | 1.4 | 36 | 81 | 16 | No | No | No | 1 |
4 | L | F | 14.7 | 1200 | 9.4 | 41 | 58 | 3 | No | No | No | 3 |
5 | L | F | 174.2 | 1350 | 5.9 | 51 | 60 | 11 | Yes | No | Yes | 0 |
6 | L | F | 12 | 1370 | 3.6 | 51 | 85 | 13 | Yes | No | No | 2 |
7 | D | F | 223.7 | 2010 | 8.9 | 25 | 30 | 28 | No | Yes | No | 3 |
8 | L | F | 23.3 | 1620 | 0.5 | 50 | 114 | 8 | No | No | Yes | — |
9 | L | F | 93.2 | 660 | 4.4 | 19.5 | 65 | 4 | No | No | Yes | 2 |
10 | L | F | 49.3 | 1380 | 1.6 | 22 | 45 | 8 | No | No | No | 1 |
11 | L | F | 71.7 | 1030 | 2.3 | 38 | 95 | 15 | No | Yes | Yes | 1 |
12 | D | F | 89.2 | 750 | 5.2 | 32 | 55 | 26 | No | Yes | No | 0 |
13 | L | F | 104.5 | 1410 | 1.1 | 57 | 106 | 13 | No | Yes | No | 0 |
14 | D | F | 232.4 | 1800 | 5.8 | 31 | 40 | 17 | No | No | No | 3 |
15 | D | F | 90.2 | 1490 | 9.7 | 42.5 | 87 | 7 | No | Yes | Yes | 1 |
16 | L | F | 46 | 1380 | 6.3 | 64 | 113 | 22 | Yes | No | Yes | 2 |
17 | D | F | 39.3 | 1330 | 0.3 | 39 | 65 | 20 | Yes | Yes | Yes | 3 |
18 | L | F | 18.3 | 1120 | 1.2 | 66 | 92 | 8 | Yes | No | No | 1 |
19 | L | F | 35 | 3000 | 2.8 | 52 | 80 | 8 | Yes | No | Yes | 1 |
20 | D | F | 13 | 900 | 2.6 | 38 | 88 | 5 | Yes | No | No | 1 |
21 | L | F | 28 | 840 | 7.9 | 27 | 60 | 13 | Yes | No | No | 2 |
22 | D | F | 8 | 1390 | 0.1 | 62 | 118 | 15 | No | No | Yes | 1 |
23 | L | F | 247 | 1290 | 2.1 | 15 | 47 | 8 | Yes | Yes | Yes | 2 |
24 | D | F | 82 | 1290 | 7.5 | 80 | 83 | 8 | No | Yes | Yes | 3 |
25 | L | F | 239 | 2700 | 0.6 | 47 | 60 | 4 | No | Yes | Yes | 0 |
26 | D | F | 180 | 1000 | 1.3 | 35 | 88 | 5 | No | No | No | 1 |
27 | D | F | 265 | 1140 | 6.1 | 41 | 98 | 13 | No | No | No | 0 |
28 | D | F | 110 | 1520 | 12.4 | 27.5 | 96 | 18 | Yes | No | Yes | 1 |
29 | L | F | 22 | 1420 | 0.5 | 12 | 70 | 20 | Yes | No | Yes | 0 |
30 | D | F | 54.5 | 1280 | 13.8 | 16 | 67 | 10 | Yes | No | No | 2 |
31 | L | F | 28 | 1260 | 1.5 | 42.5 | 133 | 9 | Yes | No | No | 2 |
32 | L | F | 38 | 2150 | 1.1 | — | 72 | 13 | No | No | Yes | 0 |
33 | L | F | 8 | 1010 | 10 | 38 | 57 | 9 | Yes | Yes | Yes | 2 |
34 | D | F | 98 | 1650 | 3.9 | 37.5 | 118 | 28 | No | No | Yes | 0 |
35 | D | F | 55 | 1100 | 3.0 | 14 | 20 | 38 | Yes | No | No | 3 |
36 | L | F | 31 | 1440 | 6.8 | 31 | 66 | 39 | Yes | No | No | 3 |
37 | D | F | 210 | 1380 | 4.2 | 53 | 89 | 39 | Yes | No | No | 3 |
38 | D | F | 220 | 1130 | 4.5 | 38 | 43 | 28 | No | Yes | Yes | 2 |
39 | D | F | 92 | 1600 | 15.3 | 40 | 55 | 32 | No | Yes | Yes | 2 |
40 | L | F | 77 | 1920 | 10.6 | 24 | 39 | 13 | No | No | Yes | 0 |
41 | L | F | 117 | 1690 | 2.4 | 49 | 67 | 10 | Yes | Yes | Yes | 3 |
42 | L | M | 17.5 | 1180 | 16 | 82 | 113 | 28 | No | No | No | 2 |
43 | L | F | 18.5 | 2000 | 0.9 | 45 | 80 | 16 | No | No | No | 1 |
44 | D | F | 71.6 | 1150 | 16.7 | 47 | 70 | 12 | No | No | Yes | 2 |
D = diffuse cutaneous; L = limited cutaneous; NGF = circulating nerve growth factor (ng/ml); ACE = circulating angiotensin converting enzyme (pg/ml/min); vWF = circulating von Willebrand factor (IU/l); DTPA = ventilation scintiscan with 99mTc radioaerosol (T/2 min); Tlco = carbon monoxide transfer factor (%); heart invol. = cardiac involvement; joint invol. = articular involvement; PNS involv. = peripheral nervous system involvement; HRCT = high resolution computed tomography (0 = normal; 1 = ground glass appearance; 2 = diffuse interstitial fibrosis; 3 = honey comb).