Table 1.
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.