Table 5 Univariable associations of hypocomplementaemia with clinical manifestations, treatment, European Scleroderma Study Group disease activity score and physician global assessments
Characteristic | Persistent normocomplementaemia (n = 859) | At least one episode of hypocomplementaemia (n = 275) |  |
|---|---|---|---|
n (%) or mean ± SD or median (IQR) | n (%) or mean ± SD or median (IQR) | p Value | |
Disease manifestationa | |||
 Raynaud’s phenomenon | 803 (93.5 %) | 262 (95.6 %) | 0.19 |
 BMI <20 kg/m2 | 104 (13.0 %) | 54 (20.5 %) | 0.0030 |
 Digital ulcers | 263 (30.7 %) | 82 (30.0 %) | 0.848 |
 Digital gangrene/amputation | 76 (8.8 %) | 17 (6.2 %) | 0.17 |
 Telangiectasia | 716 (83.6 %) | 231 (84.6 %) | 0.68 |
 Calcinosis | 340 (39.7 %) | 99 (36.3 %) | 0.31 |
 Scleroderma | 565 (66.2 %) | 198 (72.8 %) | 0.044 |
 Tendon friction rub | 88 (10.3 %) | 24 (8.8 %) | 0.47 |
 Joint contracture | 332 (38.9 %) | 99 (36.4 %) | 0.46 |
 Synovitis | 241 (28.3 %) | 82 (30.0 %) | 0.59 |
Muscle atrophy | 147 (17.2Â %) | 63 (23.2Â %) | 0.029 |
 MRSS >20 | 158 (18.7 %) | 49 (18.0 %) | 0.81 |
 Myocardial disease | 70 (8.1 %) | 17 (6.2 %) | 0.30 |
 Pericardial effusion | 45 (5.3 %) | 25 (9.1 %) | 0.023 |
 PAH | 90 (10.4 %) | 30 (10.9 %) | 0.82 |
 Pulmonary fibrosis | 263 (30.8 %) | 77 (30.1 %) | 0.40 |
 Gastrointestinal involvement | 493 (57.5 %) | 145 (52.7 %) | 0.17 |
  GAVE | 86 (10.1 %) | 27 (9.9 %) | 0.94 |
  Reflux oesophagitis | 722 (83.8 %) | 236 (85.8 %) | 0.41 |
  Oesophageal stricture | 109 (12.8 %) | 23 (8.4 %) | 0.051 |
  Oesophageal dysmotility | 346 (40.5 %) | 111 (40.8 %) | 0.92 |
  Bowel dysmotility | 226 (26.4 %) | 71 (25.9 %) | 0.88 |
  Pseudo-obstruction | 25 (2.9 %) | 12 (4.4 %) | 0.23 |
 Renal crises | 36 (4.2 %) | 8 (2.9 %) | 0.34 |
 eGFR <60 ml/minute | 229 (22.8 %) | 68 (25.0 %) | 0.55 |
 Myositis | 37 (5.2 %) | 21 (8.5 %) | 0.055 |
 CRP >8 mg/L | 266 (31.1 %) | 67 (24.9 %) | 0.051 |
 ESR >30 mm/h | 252 (29.6 %) | 71 (26.1 %) | 0.27 |
 Blood CK >200 IU/L | 105 (12.1 %) | 44 (16.0 %) | 0.098 |
 Anaemia | 302 (35.1 %) | 101 (36.7 %) | 0.62 |
 FVC <80 % | 225 (26.9 %) | 73 (27.0 %) | 0.97 |
 DLCO <80 % | 631 (80.5 %) | 201 (78.2 %) | 0.43 |
Treatmenta | |||
 Corticosteroids | 380 (43.9 %) | 126 (45.8 %) | 0.58 |
 Immunotherapy | 364 (42.1 %) | 131 (47.6 %) | 0.11 |
 Biologic therapy | 9 (1.0 %) | 7 (2.6 %) | 0.065 |
 Home oxygen | 32 (3.7 %) | 9 (3.3 %) | 0.74 |
EScSG scoreb | 2.5 (1.4) | 2 (1–4) | 0.16 |
Physician global assessmentsb | |||
 Health | 4 (3–6) | 4 (3–6) | 0.25 |
 Activity | 3 (2–5) | 3 (2–5) | 0.076 |
 Damage | 4 (2.5–6) | 4 (3–6) | 0.44 |