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. 2022 Jul 29;13:954007. doi: 10.3389/fimmu.2022.954007

Table 1.

Characteristics of SSc patients from the discovery population.

N Value
Demographic data
Females, n (%) 80 65 (81)
Age at inclusion (years), mean ± SD 80 56 ± 13
BMI (kg/m2), mean ± SD 80 24 ± 5
Diagnosis of SSc
SSc subtype 80
 Early dcSSc, n (%) 15 (19)
 Late dcSSc, n (%) 24 (30)
 lcSSc, n (%) 41 (51)
Immunological profile 80
 Anti-centromere antibodies, n (%) 20 (25)
 Anti-topoisomerase I antibodies, n (%) 21 (26)
 Anti-RNA polymerase III antibodies, n (%) 7 (9)
 Other antibody specificities, n (%) 17 (21)
 Positive ANA without antibody specificity, n (%) 6 (8)
 Negative ANA 9 (11)
Overlap with another connective tissue disease 79 25 (32)
 Systemic lupus erythematosus, n (%) 4 (5)
 Sjogren’s syndrome, n (%) 12 (15)
 Inflammatory myopathy, n (%) 4 (5)
 Rheumatoid arthritis, n (%) 2 (3)
Lymphoma (current or previous history), n (%) 80 0 (0)
Disease duration at inclusion
 Since diagnosis (years), mean ± SD 80 10 ± 10
 Since first non-Raynaud symptom (years), mean ± SD 78 11 ± 10
 Since Raynaud phenomenon onset (years), mean ± SD 76 14 ± 13
History of organ involvements
Interstitial lung disease 80
 No ILD, n (%) 26 (33)
 Limited ILD, n (%) 25 (31)
 Extensive ILD, n (%) 29 (36)
 ILD duration at inclusion (years), mean ± SD 54 7 ± 7
Pulmonary hypertension, n (%) 80 19 (24)
 Group 1, n (%) 13 (17)
 Group 1’, n (%) 1 (1)
 Group 2, n (%) 6 (8)
 Group 3, n (%) 3 (4)
 PH duration at inclusion (years), mean ± SD 19 4 ± 4
Scleroderma renal crisis, n (%) 80 0 (0)
History of digital ulcers, n (%) 80 49 (61)
Clinical evaluation at inclusion
Modified Rodnan skin score, mean ± SD 79 7.6 ± 6.5
in dcSSc patients, mean ± SD 41 10.8 ± 6.8
NYHA functional class 80
 Class I, n (%) 29 (36)
 Class II, n (%) 21 (26)
 Class III, n (%) 23 (29)
 Class IV, n (%) 7 (9)
6-minute walk distance (m), mean ± SD 74 401 ± 117
6-minute walk distance (% predicted), mean ± SD 74 72 ± 18
Active Raynaud attacks at inclusion, n (%) 76 35 (46)
Active digital ulcers at inclusion, n (%) 80 16 (20)
Telangiectasias, n (%) 79 59 (75)
Calcinosis cutis, n (%) 74 12 (16)
Biological data
ESR (mm/h), mean ± SD 74 17 ± 19
CRP (mg/L), mean ± SD 80 6.9 ± 8.9
Creatinin (mg/L), mean ± SD 80 7.9 ± 2.4
Estimated GFR (mL/min/1,73m2), mean ± SD 80 94 ± 27
Nt-pro-BNP (pg/mL), mean ± SD 80 578 ± 1452
Ferritin (ng/mL), mean ± SD 79 112 ± 121
Uric acid (mg/L), mean ± SD 80 47 ± 15
Complement activation, n (%) 80 1 (1)
Transthoracic echocardiography
Left ventricular ejection fraction (%), mean ± SD 79 64 ± 7
Left ventricular diastolic dysfunction, n (%) 78 62 (80)
Peak TRV (m/s), mean ± SD 69 2.78 ± 0.68
RA area (cm2), mean ± SD 67 15 ± 5
Pulmonary function tests
TLC (% predicted), mean ± SD 78 83 ± 19
FVC (% predicted), mean ± SD 80 88 ± 23
DLCO (% predicted), mean ± SD 77 57 ± 20
KCO (% predicted), mean ± SD 78 73 ± 21
Composite scores
EScSG-AI score, mean ± SD 78 1.58 ± 1.31
 in lcSSc patients, mean ± SD 41 1.46 ± 1.22
 in dcSSc patients, mean ± SD 37 1.72 ± 1.40
Medsger severity score, mean ± SD 77 5.90 ± 2.86
sHAQ score, mean ± SD 72 0.90 ± 0.63
Treatments at inclusion
Corticosteroids, n (%) 80 47 (59)
 Corticosteroid dosage (mg prednisone equivalent/day), mean ± SD 47 8 ± 3
Immunosuppressants, n (%) 80 43 (54)
 Cyclophosphamide, n (%) 0 (0)
 Mycophenolate mofetil, n (%) 31 (39)
 Methotrexate, n (%) 3 (4)
 Azathioprine, n (%) 2 (3)
 Rituximab (current or previous), n (%) 7 (9)
 Current treatment by rituximab (last infusion < 12 months) 3 (4)
Hydroxychloroquine, n (%) 80 7 (8.8)
PAH specific drugs*, n (%) 80 21 (26)

ANA, antinuclear antibodies; BMI, body mass index; CRP, C-reactive protein; dc, diffuse cutaneous; DLCO, diffusing capacity of the lung for carbon monoxide; EScSG-AI, European Scleroderma Study Group Activity Index; ESR, erythrocyte sedimentation rate; FVC, forced vital capacity; GFR, glomerular filtration rate; sHAQ, scleroderma Health Assessment Questionnaire; ILD, interstitial lung disease; KCO, diffusing coefficient for carbon monoxide; lc, limited cutaneous; Nt-pro-BNP, N-terminal prohormone of brain natriuretic peptide; NYHA, New York Heart Association; PAH, pulmonary arterial hypertension; RA, right atrium; SSc, systemic sclerosis; TLC, total lung capacity; TRV, tricuspid regurgitation velocity.

*PAH specific drugs included phosphodiesterase 5 inhibitors, endothelin receptor antagonists and prostacyclin analogues. These could have been prescribed for PAH, digital ulcers and/or refractory Raynaud phenomenon.