Table 1.
Baseline characteristics of the study population (n = 334)
57.9 (13.6)
| Baseline characteristics | All patients | ‘Non-scleroderma pattern’ | ‘Scleroderma pattern’ | ‘Early’ | ‘Active’ | ‘Late’ |
|---|---|---|---|---|---|---|
| (n = 334) | (n = 92) | (n = 242) | (n = 53) | (n = 170) | (n = 19) | |
| Age, mean (s.d.), years | 59.03 (12.9) | 57.4 (13.9) | 56.2 (14.2) | 57.2 (14.0) | 62.7 (10.9) | |
| Gender | ||||||
| male, n (%) | 69 (20.7) | 17 (18.5) | 52 (21.5) | 7 (13.2) | 40 (23.5) | 5 (26.3) |
| female, n (%) | 265 (79.3) | 75 (81.5) | 190 (78.5) | 46 (86.6) | 130 (76.5) | 14 (73.7) |
| Disease duration (years), median (IQR) | 5 (1–12) | 5 (2–14) | 5 (1–11) | 4 (0–9) | 5 (1–13) | 9 (2–13) |
| SSc classification criteria | ||||||
| ACR criteria, n (%) | 277 (82.9) | 76 (82.6) | 201 (83.1) | 39 (73.6) | 145 (85.3) | 17 (89.5) |
| 2013 ACR/EULAR criteria, n (%) | 302 (90.4) | 78 (84.8) | 224 (92.6) | 47 (88.7) | 158 (92.9) | 19 (100.0) |
| LeRoy subset | ||||||
| lSSc, n (%) | 18 (5.4) | 6 (6.5) | 12 (5.0) | 7 (13.2) | 5 (2.9) | 0 (0.0) |
| lcSSc, n (%) | 203 (60.8) | 51 (55.4) | 152 (62.8) | 33 (62.3) | 111 (65.3) | 8 (42.1) |
| dcSSc, n (%) | 113 (33.8) | 35 (38.0) | 78 (32.2) | 13 (24.5) | 54 (31.8) | 11 (57.9) |
| SSc-specific antibodies | 231 (69.2) | 65 (70.7) | 166 (68.6) | 33 (62.3) | 120 (70.6) | 13 (68.4) |
| ACA, n (%) | 115 (34.4) | 28 (30.4) | 87 (36.0) | 19 (35.8) | 64 (37.6) | 4 (21.1) |
| Anti-topo-1 (Scl-70) antibodies, n (%) | 99 (29.6) | 28 (30.4) | 171 (29.3) | 11 (20.8) | 52 (30.6) | 8 (42.1) |
| Anti-RNA polymerase III antibodies, n (%) | 17 (5.1) | 9 (9.8) | 8 (3.3) | 3 (5.7) | 54 (2.4) | 1 (5.3) |
| NVC parameters | ||||||
| Quantitative assessment | ||||||
| Capillary density | ||||||
| mean number of capillaries/mm, mean (s.d.) | 5.4 (1.6) | 6.4 (1.7) | 5.1 (1.3) | 6.9 (0.8) | 4.6 (0.9) | 4.0 (1.2) |
| Capillary dimension | ||||||
| number of patients with giant capillaries, n (%) | 223 (66.8) | 0 (0.0) | 223 (92.1) | 53 (100.0) | 170 (100.0) | 0 (0.0) |
| mean number of giant capillaries/mm, mean (s.d.) | 0.4 (0.5) | 0.0 (0.0) | 0.5 (0.5) | 0.4 (0.4) | 0.6 (0.5) | 0.0 (0.0) |
| Capillary morphology | ||||||
| number of patients with abnormally shaped capillaries, n (%) | 311 (93.1) | 83 (90.2) | 228 (94.2) | 51 (96.2) | 158 (92.9) | 19 (100.0) |
| mean number of abnormally shaped capillaries/mm, mean (s.d.) | 0.9 (0.7) | 0.9 (0.7) | 0.9 (0.7) | 0.7 (0.5) | 0.8 (0.7) | 1.9 (0.6) |
| Microhaemorrhages | ||||||
| number of patients with microhaemorrhages, n (%) | 197 (59.0) | 31 (33.7) | 166 (68.6) | 37 (69.8) | 121 (71.2) | 8 (42.1) |
| mean number of microhaemorrhages/mm, mean (s.d.) | 0.2 (0.2) | 0.1 (0.1) | 0.2 (0.2) | 0.2 (0.2) | 0.2 (0.2) | 0.1 (0.1) |
| Qualitative assessment | ||||||
| ‘Non-scleroderma pattern’ | 92 (27.5) | 92 (100.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) |
| ‘Scleroderma pattern’ | 242 (72.5) | 0 (0.0) | 242 (100.0) | 53 (100.0) | 170 (100.0) | 19 (100.0) |
| Early NVC pattern | 53 (21.9) | 0 (0.0) | 53 (21.9) | 53 (100.0) | 0 (0.0) | 0 (0.0) |
| Active NVC pattern | 170 (70.2) | 0 (0.0) | 170 (70.2) | 0 (0.0) | 170 (100.0) | 0 (0.0) |
| Late NVC pattern | 19 (7.9) | 0 (0.0) | 19 (7.9) | 0 (0.0) | 0 (0.0) | 19 (100.0) |
| Severe NVC patterna | 189 (78.1) | 0 (0.0) | 189 (78.1) | 0 (0.0) | 170 (90.0) | 19 (10.0) |
| Peripheral vascular involvement | 160 (47.9) | 35 (38.0) | 125 (51.7) | 22 (41.5) | 92 (54.1) | 11 (57.9) |
| History | 129 (38.6) | 29 (31.5) | 100 (41.3) | 16 (30.2) | 76 (44.7) | 8 (42.1) |
| History of digital ulcers, n (%) | 112 (33.5) | 26 (28.3) | 86 (35.5) | 12 (22.6) | 66 (38.8) | 8 (42.1) |
| History of gangrene, n (%) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) |
| History of pitting scars, n (%) | 28 (8.4) | 7 (7.6) | 7 (7.6) | 4 (7.5) | 17 (10.0) | 0 (0.0) |
| Active | 130 (38.9) | 27 (29.3) | 103 (42.6) | 17 (32.1) | 76 (44.7) | 10 (52.6) |
| Active digital ulcers, n (%) | 75 (22.5) | 18 (19.6) | 57 (23.6) | 11 (20.8) | 38 (22.4) | 8 (42.1) |
| Active gangrene, n (%) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) |
| Active pitting scars, n (%) | 63 (18.9) | 12 (13.0) | 51 (21.1) | 7 (13.2) | 42 (24.7) | 2 (10.5) |
| Pulmonary involvement | ||||||
| ILD, n (%) | 149 (44.6) | 43 (46.7) | 106 (43.8) | 22 (41.5) | 75 (44.1) | 9 (47.4) |
| Vascular lung involvement | ||||||
| PH, n (%) | 62 (18.6) | 16 (17.4) | 46 (19.0) | 4 (7.5) | 36 (21.2) | 6 (31.6) |
| PAH, n (%) | 2 (0.6) | 1 (1.1) | 1 (0.4) | 0 (0.0) | 1 (0.6) | 0 (0.0) |
| Skin | ||||||
| mRSS, mean (s.d.) | 8.8 (6.3) | 8.9 (6.7) | 8.7 (6.2) | 6.9 (4.7) | 8.9 (5.9) | 11.3 (10.3) |
| Scleredema, n (%) | 103 (30.8) | 23 (25.0) | 80 (33.1) | 18 (34.0) | 59 (34.7) | 3 (15.8) |
| Heart | 12 (3.6) | 4 (4.3) | 8 (3.3) | 1 (1.9) | 5 (2.9) | 2 (10.5) |
| LVEF < 50%, n (%) | 2 (0.6) | 1 (1.1) | 1 (0.4) | 0 (0.0) | 1 (0.6) | 0 (0.0) |
| Pericardial effusion, n (%) | 10 (3.0) | 3 (3.3) | 7 (2.9) | 1 (1.9) | 4 (2.4) | 2 (10.5) |
| Gastrointestinal tract | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) |
| Intestinal obstruction, n (%) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) |
| Malabsorption, n (%) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) |
| SRC, n (%) | 2 (0.6) | 1 (1.1) | 1 (0.4) | 0 (0.0) | 1 (0.6) | 0 (0.0) |
| Musculoskeletal involvement | 68 (20.4) | 19 (20.7) | 49 (20.2) | 7 (13.2) | 35 (20.6) | 7 (36.8) |
| Synovitis, n (%) | 37 (11.1) | 13 (14.1) | 24 (9.9) | 1 (1.9) | 18 (10.6) | 5 (26.3) |
| Muscle weakness, n (%) | 27 (8.1) | 8 (8.7) | 19 (7.9) | 4 (7.5) | 14 (8.2) | 1 (5.3) |
| CK elevation, n (%) | 23 (6.9) | 7 (7.6) | 16 (6.6) | 4 (7.5) | 11 (6.5) | 1 (5.3) |
| Medication | ||||||
| Vasodilator therapy, n (%) | 232 (69.5) | 58 (63.0) | 174 (71.9) | 34 (64.2) | 126 (74.1) | 14 (73.7) |
| Prostacyclin, n (%) | 86 (25.7) | 18 (19.6) | 68 (28.1) | 10 (18.9) | 54 (31.8) | 4 (21.1) |
| Calcium antagonists, n (%) | 192 (57.5) | 49 (53.3) | 143 (59.4) | 29 (54.7) | 101 (59.4) | 13 (68.4) |
| Endothelin receptor antagonists, n (%) | 47 (14.1) | 13 (14.1) | 34 (14.0) | 4 (7.5) | 28 (16.5) | 2 (10.5) |
| Phosphodiesterase type 5 inhibitor, n (%) | 14 (4.2) | 2 (2.2) | 12 (5.0) | 1 (1.9) | 9 (5.3) | 2 (10.5) |
| Immunosuppressive therapy | 104 (31.1) | 34 (37.0) | 70 (28.9) | 15 (28.3) | 46 (27.1) | 9 (47.4) |
| MTX, n (%) | 60 (18.0) | 20 (21.7) | 40 (16.5) | 8 (15.1) | 27 (15.9) | 5 (26.3) |
| MMF, n (%) | 20 (6.0) | 7 (7.6) | 13 (5.4) | 3 (5.7) | 9 (5.3) | 1 (5.3) |
| CYC, n (%) | 4 (1.2) | 3 (3.3) | 1 (0.4) | 0 (0.0) | 0 (0.0) | 1 (5.3) |
| Rituximab, n (%) | 16 (4.8) | 6 (6.5) | 10 (4.1) | 1 (1.9) | 5 (2.9) | 4 (21.1) |
| AZA, n (%) | 13 (3.9) | 3 (3.3) | 10 (4.1) | 3 (5.7) | 7 (4.1) | 0 (0.0) |
| Autologous stem cell transplantation, n (%) | 2 (0.6) | 0 (0.0) | 2 (0.8) | 0 (0.0) | 2 (1.2) | 0 (0.0) |
| Renal therapy, n (%) | 41 (12.3) | 12 (13.0) | 29 (12.0) | 4 (7.5) | 21 (12.4) | 4 (21.1) |
| ACE-inhibitor, n (%) | 41 (12.3) | 12 (13.0) | 29 (12.0) | 4 (7.5) | 21 (12.4) | 4 (21.1) |
| Dialysis, n (%) | 1 (0.3) | 0 (0.0) | 1 (0.4) | 0 (0.0) | 1 (0.6) | 0 (0.0) |
a
‘Active’ and ‘late’ NVC patterns were considered ‘severe’ NVC patterns. ACE: angiotensin-converting enzyme; ILD: interstitial lung disease; IQR: interquartile range; lSSc: limited SSc; LVEF: left ventricular ejection fraction; mRSS: modified Rodnan skin score; NVC: nailfold videocapillaroscopy; PAH: pulmonary arterial hypertension; PH: pulmonary hypertension; SRC: scleroderma renal crisis.