Table 2.
Demographics and disease characteristics of the 5 mRSS trajectories classes (n = 198)
| No. with available data | Class 1 (n = 117) |
Class 2 (n = 43) |
Class 3 (n = 13) |
Class 4 (n = 13) |
Class 5 (n = 12) |
p | |
|---|---|---|---|---|---|---|---|
| Demographics | |||||||
| Sex, female, no. (%) | 198 | 93/117 (79.5) | 28/43 (65.1) | 9/13 (69.2) | 7/13 (53.9) | 8/12 (66.7) | 0.13 |
| Ethnicity, no. (%) | <.001 | ||||||
| White | 161 | 88/98 (89.8) | 28/32 (87.5) | 8/11 (72.7) | 8/11 (72.7) | 8/9 (88.9) | |
| Black | 161 | 6/98 (6.1) | 3/32 (9.4) | 3/11 (27.3) | 3/11 (27.3) | 1/9 (11.1) | |
| Asian | 161 | 4/98 (4.1) | 1/32 (3.1) | 0/11 (0.0) | 0/11 (0.0) | 0/9 (0.0) | |
| Age, mean ± SD, years | 198 | 52.9 ± 13.9 | 49.2 ± 14.1 | 43.6 ± 17.0 | 47.8 ± 14.6 | 51.9 ± 14.5 | 0.15 |
| Disease characteristics | |||||||
| Disease duration, median (IQR), years | 198 | 0.8 (0.4; 1.3) | 0.9 (0.5; 1.2) | 0.9 (0.5; 1.1) | 0.6 (0.2; 0.9) | 1.1 (0.4; 1.3) | 0.21 |
| Duration from RP, median (IQR), years | 187 | 1.6 (0.6; 6.0) | 1.3 (0.6; 1.7) | 0.8 (0.8; 2.0) | 0.6 (0.2; 0.9) | 1.3 (0.4; 3.3) | 0.018 |
| Follow-up, mean ± SD, years | 198 | 4.9 ± 0.2 | 5.0 ± 0.6 | 6.8 ± 1.0 | 7.4 ± 1.5 | 4.1 ± 0.4 | 0.10 |
| Antibodies a, no. (%) | |||||||
| Anti-nuclear | 165 | 89/93 (95.7) | 37/39 (94.8) | 9/11 (81.8) | 11/12 (91.7) | 10/10 (100) | 0.32 |
| Anti-centromere | 152 | 41/97 (42.3) | 1/28 (3.6) | 0/9 (0.0) | 1/10 (10.0) | 0/8 (0.0) | <.001 |
| Anti-topoisomerase I | 152 | 41/97 (42.3) | 21/28 (75.0) | 8/9 (88.9) | 8/10 (80.0) | 7/8 (87.5) | <.001 |
| Anti-RNAP3 | 152 | 3/97 (3.1) | 4/28 (14.3) | 0/9 (0.0) | 0/10 (0.0) | 1/8 (12.5) | 0.11 |
| Anti-U1RNP | 152 | 7/97 (7.2) | 2/28 (7.1) | 0/9 (0.0) | 0/10 (0.0) | 0/8 (0.0) | NA |
| Anti-PM/Scl | 152 | 5/97 (5.2) | 1/28 (3.6) | 0/9 (0.0) | 0/10 (0.0) | 0/8 (0.0) | NA |
| Only others | 152 | 4/97 (4.1) | 1/28 (3.6) | 1/9 (11.1) | 2/8 (20.0) | 0/8 (0.0) | 0.19 |
| Skin variables | |||||||
| Cutaneous subset, limited, no (%) | 195 | 95/115 (82.6) | 1/42 (2.4) | 2/13 (15.4) | 0/13 (0.0) | 0/12 (0.0) | <.001 |
| STPR, median (IQR); units per years | 198 | 3.9 (1.2; 9.3) | 21.8 (16.0; 35.9) | 7.8 (5.7; 13.4) | 38.5 (29.0; 131.3) | 34.4 (27.6; 74.9) | <.001 |
| mRSS trajectories, [95% CI] | |||||||
| Mean baseline mRSS | 198 | 4.1 [3.2; 5.0] | 20.8 [19.0; 22.5] | 8.7 [6.0; 11.5] | 25.1 [22.6; 27.6] | 35.1 [32.2; 37.9] | NA |
| Mean mRSS at 6 months | 198 | 4.3 [3.3; 5.4] | 19.5 [17.7; 21.3] | 14.5 [11.4; 17.6] | 31.9 [28.7; 35.1] | 31.1 [27.6; 34.6] | NA |
| Mean mRSS at 1 year | 198 | 4.6 [3.3; 5.9] | 18.4 [16.3; 20.5] | 18.7 [14.8; 22.7] | 36.9 [32.7; 41.0] | 27.6 [23.3; 31.8] | NA |
| Mean mRSS at 2 years | 198 | 5.1 [3.7; 6.4] | 16.8 [14.5; 19.0] | 23.1 [18.5; 27.6] | 41.5 [37.0; 46.1] | 21.5 [17.2; 25.7] | NA |
| Mean mRSS at 3 years | 198 | 5.6 [4.3; 6.9] | 15.7 [13.6; 17.9] | 21.6 [17.8; 25.5] | 39.1 [35.3; 42.8] | 16.8 [10.1; 23.5] | NA |
| Mean mRSS at 4 years | 198 | 6.2 [3.8; 8.6] | 15.4 [11.0; 19.8] | 14.5 [8.4; 20.7] | 29.5 [22.7; 36.2] | 13.5 [0; 29.5] | NA |
| Baseline organ involvement, no. (%) | |||||||
| Telangiectasia | 183 | 48/109 (44.0) | 15/38 (39.5) | 4/12 (33.3) | 4/13 (30.8) | 5/11 (45.5) | 0.85 |
| Calcinosis | 175 | 13/104 (12.5) | 3/37 (8.1) | 2/11 (18.2) | 2/12 (16.7) | 0/11 (0.0) | 0.57 |
| Joints | 191 | 53/114 (46.5) | 31/40 (77.5) | 11/13 (84.6) | 10/13 (76.9) | 9/11 (81.8) | <.001 |
| Muscles | 194 | 20/117 (17.1) | 19/40 (47.5) | 5/13 (38.5) | 5/13 (38.5) | 4/11 (36.4) | 0.001 |
| Digital ulcers | 181 | 33/111 (29.7) | 19/34 (55.9) | 9/13 (69.2) | 6/12 (50.0) | 9/11 (81.8) | <.001 |
| Gastrointestinal tracts | 187 | 51/111 (46.0) | 25/40 (62.5) | 9/12 (75.0) | 9/12 (75.0) | 5/12 (41.7) | 0.062 |
| Interstitial lung disease | 181 | 34/106 (32.1) | 19/40 (47.5) | 8/12 (66.7) | 7/11 (63.6) | 4/12 (33.3) | 0.040 |
| FVC, median % (IQR) | 160 | 101 (86.0; 111.0) | 86.0 (70.8; 102.5) | 88.5 (75;0; 111.5) | 88.0 (68.5; 93.0) | 82.0 (55.0; 102.0) | 0.009 |
| DLCO, median % (IQR) | 155 | 66.0 (50.0; 80.0) | 56 (43.0; 69.0) | 67.0 (53.0; 72.0) | 65.0 (47.0; 86.9) | 61.0 (42.0; 69.0) | 0.35 |
| Heart | 187 | 7/109 (6.4) | 3/41 (7.3) | 2/13 (15.4) | 2/12 (16.7) | 1/12 (8.3) | 0.40 |
| Pulmonary hypertension | 194 | 10/114 (8.8) | 2/42 (4.8) | 1/13 (7.7) | 2/13 (15.4) | 0/12 (0.0) | 0.58 |
| Renal crisis | 123 | 1/59 (1.7) | 7/31 (22.6) | 1/11 (9.1) | 3/12 (25.0) | 0/10 (0.0) | 0.003 |
| Biological variable, no. (%) | |||||||
| Baseline CRP level, ≥ 6 mg/L | 148 | 19/85 (22.4) | 15/32 (46.9) | 5/11 (45.5) | 7/10 (70.0) | 5/10 (50.0) | 0.003 |
| Treatments b, no. (%) | |||||||
| Steroids and/or IS | 185 | 57/107 (53.3) | 33/40 (82.5) | 13/13 (100) | 12/13 (92.3) | 12/12 (100.0) | <.001 |
| Steroids | 181 | 54/108 (50.0) | 29/38 (76.3) | 11/12 (91.7) | 9/12 (75.0) | 9/11 (81.8) | 0.002 |
| Methotrexate | 166 | 13/103 (12.6) | 10/32 (31.3) | 3/13 (23.1) | 2/9 (22.2) | 4/9 (44.4) | 0.028 |
| Azathioprine | 160 | 6/100 (6.0) | 5/31 (16.1) | 2/11 (18.2) | 3/9 (33.3) | 3/9 (33.3) | 0.008 |
| Mycophenolate mofetil | 170 | 21/103 (20.4) | 15/34 (44.1) | 5/12 (41.7) | 8/11 (72.7) | 9/10 (90.0) | <.001 |
| Cyclophosphamide | 174 | 18/102 (17.7) | 17/36 (47.2) | 6/13 (46.2) | 8/12 (66.7) | 6/11 (54.6) | <.001 |
| Rituximab | 153 | 4/99 (4.0) | 0/27 (0.0) | 1/11 (9.1) | 1/7 (14.3) | 1/9 (11.1) | NA |
Anti-RNAP3 anti-RNA polymerase III antibodies, CRP C-reactive protein, disease duration duration from the first non-RP symptom, DLCO diffusing capacity of the lung for carbon monoxide (% of predicted value), FVC forced vital capacity (% of predicted value), IS immunosuppressive treatment, NA not applicable, RP Raynaud’s phenomenon, STPR skin thickening progression rate
aThe sum of % may be different from 100% because some patients had either unidentified ANA or multiple autoantibodies
bDuring follow-up