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Table 2 Disease manifestations in cohort (n = 1140)

From: The association of low complement with disease activity in systemic sclerosis: a prospective cohort study

Characteristics n (%) or median (25th–75th IQR)
Disease manifestationa
 Raynaud’s phenomenon 1065 (94.0 %)
 Digital ulcers 345 (30.5 %)
 Digital gangrene/amputation 93 (8.2 %)
 Telangiectasia 947 (83.8 %)
 Calcinosis 439 (38.9 %)
 Scleroderma 763 (67.8 %)
 Tendon friction rub 112 (10.0 %)
 Joint contracture 431 (38.3 %)
 Synovitis 323 (28.7 %)
 Muscle atrophy 210 (18.7 %)
 MRSS score >20 207 (18.5 %)
 Myocardial disease 87 (7.6 %)
 Pericardial effusionb 70 (6.2 %)
 PAHc 298 (26.9 %)
 ILDd 340 (30.1 %)
 Gastrointestinal involvemente 638 (56.3 %)
  GAVE 113 (10.0 %)
  Reflux oesophagitis 958 (84.3 %)
  Oesophageal stricture 207 (18.3 %)
  Oesophageal dysmotility 457 (40.6 %)
  Bowel dysmotility 297 (26.3 %)
  Pseudo-obstruction 37 (3.3 %)
 Renal crisesf 44 (3.9 %)
 eGFR <60 ml/minute 297 (26.4 %)
 Myositisg 58 (6.0 %)
 CRP >8 mg/L 333 (29.7 %)
 ESR >30 mm/h 323 (28.8 %)
 Blood CK >200 IU/L 122 (11.0 %)
 Anaemiah 403 (35.5 %)
 FVC <80 % predicted 298 (26.9 %)
 DLCO <80 % predicted 832 (79.9 %)
EScSG disease activity scorei 2.5 (1–4)
Treatmentsa
 Corticosteroids 506 (44.4 %)
 Immunotherapyj 495 (43.4 %)
 Biological therapyk 16 (1.4 %)
 Home oxygen 41 (3.6 %)
Physician global assessmentsl
 Overall healthm 4 (3–6)
 Activityn 3 (2–5)
 Damagen 4 (3–6)
  1. Abbreviations: MRSS Modified Rodnan skin score, EScSG European Scleroderma Study Group, PAH Pulmonary arterial hypertension, ILD Interstitial lung disease, GAVE Gastric antral vascular ectasia, eGFR Estimated glomerular filtration rate, CRP C-reactive protein, ESR Erythrocyte sedimentation rate, CK Creatinine kinase, FVC Forced vital capacity, DLCO Diffusing capacity of the lung for carbon monoxide corrected for haemoglobin
  2. aEver from disease onset to most recent visit
  3. bPericardial effusion defined by echocardiography
  4. cPAH defined by right heart catheterization with a mean pulmonary artery pressure ≥25 mmHg and a pulmonary arterial wedge pressure ≤15 mmHg
  5. dPulmonary fibrosis defined by chest high-resolution computed tomography
  6. eGastrointestinal symptoms defined as the presence of any of reflux, dysphagia, post-prandial bloating, vomiting, constipation, diarrhoea or anal incontinence as defined in text
  7. fRenal crisis defined as the presence of at least two of the following: new-onset hypertension, rising creatinine or microangiopathic haemolytic anaemia
  8. gMyositis defined as either definite (biopsy), suspected (CK or electromyogram) or possible (magnetic resonance imaging scan)
  9. hAnaemia defined as haemoglobin level <135 g/L in males and <120 g/L in females
  10. iCalculated without hypocomplementaemia. Final scores range from 0 to 9, with higher scores indicating higher disease activity
  11. jIncludes leflunomide, methotrexate, azathioprine, penicillamine, hydroxychloroquine, mycophenolate, cyclophosphamide and calcineurin inhibitors
  12. kIncludes tumour necrosis factor alpha inhibitors, tocilizumab, abatacept, anti-CD20 antibodies and other B-cell modulators
  13. lHighest score ever recorded over the study period
  14. mScores range from 0 to 10, with higher scores being indicative of worse overall health
  15. nScores range from 0 to 10, with higher scores being indicative of higher disease activity or damage