Table 2.
Definitions of SSc-ILD progression based on a single parameter
| SSc mixed cohorts | SSc-ILD cohorts | ||||||||
| Time, months (n papers) |
n patients (n papers) | n ILD patients (n papers) | n progressors (n papers) |
References | Time frame, months (n papers) |
n patients (n papers) | n progressors (n papers) | References | |
| FVC | |||||||||
| Decline FVC>15% | 6 (1) 65 (1) |
200 (2) | 84 (2) | 19 (2) | 167 168 | 12 (2) 60 (1) |
253 (3) | 26 (3) | 29 169 170 |
| Decline FVC>10% | 12 (9) 24 (4) 36 (4) 48 (1) >60 (3) Unk (3) |
11 604 (22) | 2746 (20) | 976 (19) | 20–27 30 71 116 171–179 | 6 (3) 12 (5) 24 (6) >60 (4) |
3007 (18) | 424 (16) | 28 29 74 75 125 150 161 180–190 |
| Decline FVC>5% | 12 (1) Unk (1) |
6527 (2) | 100 (1) | 401 (2) | 191 192 | 12 (4) 24 (3) Unk (1) |
1777 (8) | 589 (6) | 28 124 188 189 193–196 |
| Decline FVC>3.3% | – | – | – | – | – | 12 (1) 48 (1) 52 (1) Unk (1) |
1418 (4) | 571 (3) | 197–200 |
| DLCO | |||||||||
| Decline DLCO>15% | 12 (2) 24 (1) 60 (2) |
568 (5) | 445 (5) | 135 (5) | 24 118 172 173 191 | 6 (2) 12 (2) 36 (1) 60 (2) |
505 (7) | 168 (7) | 29 85 161 170 183 201 202 |
| Decline DLCO>10% | 12 (2) 55 (1) >60 (1) Unk (1) |
340 (5) | 112 (2) | 86 (5) | 167 171 176 203 204 | 24 (2) | 30 (2) | 9 (1) | 140 195 |
| HRCT | |||||||||
| Increase ILD extent>10% | 12 (2) | 347 (2) | 213 (2) | 6 (2) | 30 205 | 24 (2) | 145 (2) | 5 (2) | 31 32 |
| Increased ILD extent>20% | 24 (1) Unk (1) |
22 (2) | 8 (2) | 206 207 | |||||
| Increase ILD extent | 12 (1) 24 (1) 37 (1) 62 (1) |
1103 (4) | 480 (4) | 105 (4) | 148 208–210 | 12 (1) 24 (1) |
217 (2) | 34 (1) | 211 212 |
DLCO, carbon monoxide diffusing capacity; FVC, forced vital capacity; HRCT, high-resolution CT; ILD, interstitial lung disease.