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Table 2 Demographics and disease characteristics of the 5 mRSS trajectories classes (n = 198)

From: Early trajectories of skin thickening are associated with severity and mortality in systemic sclerosis

 No. with available dataClass 1
(n = 117)
Class 2
(n = 43)
Class 3
(n = 13)
Class 4
(n = 13)
Class 5
(n = 12)
p
Demographics
 Sex, female, no. (%)19893/117 (79.5)28/43 (65.1)9/13 (69.2)7/13 (53.9)8/12 (66.7)0.13
 Ethnicity, no. (%)<.001
  White16188/98 (89.8)28/32 (87.5)8/11 (72.7)8/11 (72.7)8/9 (88.9) 
  Black1616/98 (6.1)3/32 (9.4)3/11 (27.3)3/11 (27.3)1/9 (11.1)
  Asian1614/98 (4.1)1/32 (3.1)0/11 (0.0)0/11 (0.0)0/9 (0.0)
 Age, mean ± SD, years19852.9 ± 13.949.2 ± 14.143.6 ± 17.047.8 ± 14.651.9 ± 14.50.15
Disease characteristics
 Disease duration, median (IQR), years1980.8 (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), years1871.6 (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, years1984.9 ± 0.25.0 ± 0.66.8 ± 1.07.4 ± 1.54.1 ± 0.40.10
 Antibodies a, no. (%)
  Anti-nuclear16589/93 (95.7)37/39 (94.8)9/11 (81.8)11/12 (91.7)10/10 (100)0.32
  Anti-centromere15241/97 (42.3)1/28 (3.6)0/9 (0.0)1/10 (10.0)0/8 (0.0)<.001
  Anti-topoisomerase I15241/97 (42.3)21/28 (75.0)8/9 (88.9)8/10 (80.0)7/8 (87.5)<.001
  Anti-RNAP31523/97 (3.1)4/28 (14.3)0/9 (0.0)0/10 (0.0)1/8 (12.5)0.11
  Anti-U1RNP1527/97 (7.2)2/28 (7.1)0/9 (0.0)0/10 (0.0)0/8 (0.0)NA
  Anti-PM/Scl1525/97 (5.2)1/28 (3.6)0/9 (0.0)0/10 (0.0)0/8 (0.0)NA
  Only others1524/97 (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 (%)19595/115 (82.6)1/42 (2.4)2/13 (15.4)0/13 (0.0)0/12 (0.0)<.001
 STPR, median (IQR); units per years1983.9 (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 mRSS1984.1 [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 months1984.3 [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 year1984.6 [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 years1985.1 [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 years1985.6 [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 years1986.2 [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. (%)
 Telangiectasia18348/109 (44.0)15/38 (39.5)4/12 (33.3)4/13 (30.8)5/11 (45.5)0.85
 Calcinosis17513/104 (12.5)3/37 (8.1)2/11 (18.2)2/12 (16.7)0/11 (0.0)0.57
 Joints19153/114 (46.5)31/40 (77.5)11/13 (84.6)10/13 (76.9)9/11 (81.8)<.001
 Muscles19420/117 (17.1)19/40 (47.5)5/13 (38.5)5/13 (38.5)4/11 (36.4)0.001
 Digital ulcers18133/111 (29.7)19/34 (55.9)9/13 (69.2)6/12 (50.0)9/11 (81.8)<.001
 Gastrointestinal tracts18751/111 (46.0)25/40 (62.5)9/12 (75.0)9/12 (75.0)5/12 (41.7)0.062
 Interstitial lung disease18134/106 (32.1)19/40 (47.5)8/12 (66.7)7/11 (63.6)4/12 (33.3)0.040
 FVC, median % (IQR)160101 (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)15566.0 (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
 Heart1877/109 (6.4)3/41 (7.3)2/13 (15.4)2/12 (16.7)1/12 (8.3)0.40
 Pulmonary hypertension19410/114 (8.8)2/42 (4.8)1/13 (7.7)2/13 (15.4)0/12 (0.0)0.58
 Renal crisis1231/59 (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/L14819/85 (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 IS18557/107 (53.3)33/40 (82.5)13/13 (100)12/13 (92.3)12/12 (100.0)<.001
  Steroids18154/108 (50.0)29/38 (76.3)11/12 (91.7)9/12 (75.0)9/11 (81.8)0.002
  Methotrexate16613/103 (12.6)10/32 (31.3)3/13 (23.1)2/9 (22.2)4/9 (44.4)0.028
  Azathioprine1606/100 (6.0)5/31 (16.1)2/11 (18.2)3/9 (33.3)3/9 (33.3)0.008
  Mycophenolate mofetil17021/103 (20.4)15/34 (44.1)5/12 (41.7)8/11 (72.7)9/10 (90.0)<.001
  Cyclophosphamide17418/102 (17.7)17/36 (47.2)6/13 (46.2)8/12 (66.7)6/11 (54.6)<.001
  Rituximab1534/99 (4.0)0/27 (0.0)1/11 (9.1)1/7 (14.3)1/9 (11.1)NA
  1. 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
  2. aThe sum of % may be different from 100% because some patients had either unidentified ANA or multiple autoantibodies
  3. bDuring follow-up
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