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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
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Arthritis Research & Therapy

ISSN: 1478-6362

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