|
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)
|
-
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
-
aEver from disease onset to most recent visit
-
bPericardial effusion defined by echocardiography
-
cPAH defined by right heart catheterization with a mean pulmonary artery pressure ≥25 mmHg and a pulmonary arterial wedge pressure ≤15 mmHg
-
dPulmonary fibrosis defined by chest high-resolution computed tomography
-
eGastrointestinal symptoms defined as the presence of any of reflux, dysphagia, post-prandial bloating, vomiting, constipation, diarrhoea or anal incontinence as defined in text
-
fRenal crisis defined as the presence of at least two of the following: new-onset hypertension, rising creatinine or microangiopathic haemolytic anaemia
-
gMyositis defined as either definite (biopsy), suspected (CK or electromyogram) or possible (magnetic resonance imaging scan)
-
hAnaemia defined as haemoglobin level <135 g/L in males and <120 g/L in females
-
iCalculated without hypocomplementaemia. Final scores range from 0 to 9, with higher scores indicating higher disease activity
-
jIncludes leflunomide, methotrexate, azathioprine, penicillamine, hydroxychloroquine, mycophenolate, cyclophosphamide and calcineurin inhibitors
-
kIncludes tumour necrosis factor alpha inhibitors, tocilizumab, abatacept, anti-CD20 antibodies and other B-cell modulators
-
lHighest score ever recorded over the study period
-
mScores range from 0 to 10, with higher scores being indicative of worse overall health
-
nScores range from 0 to 10, with higher scores being indicative of higher disease activity or damage
