N = 234 | Baseline | Followup | p |
---|---|---|---|
Age, mean ± SD | 52 ± 14 | — | — |
Female, n (%) | 196 (84) | — | — |
Cardiovascular medication, n (%) | |||
ACEi/ARB | 135 (38) | — | — |
Beta-blocker | 29 (8) | — | — |
Ca2+ channel-blocker | 155 (44) | — | — |
Diuretics | 49 (14) | — | — |
Immunosuppressive medication, n (%) | |||
Corticosteroids | 30 (9) | — | — |
Cyclophosphamide | 4 (1) | — | — |
Methotrexate | 32 (9) | — | — |
Azathioprine | 12 (3) | — | — |
mRSS, median (IQR) | 3 (0–6) | 4 (0–6) | 0.728 |
mRSS > 15, n (%) | 20 (9) | 16 (7) | < 0.001 |
Arthritis, n (%) | 23 (10) | 15 (6) | 0.441 |
Proximal muscle weakness, n (%) | 31 (13) | 34 (15) | 0.210 |
Lung fibrosis, n (%) | 83 (36) | 95 (41) | < 0.001 |
6-min walking distance, m, mean ± SD | 513 ± 128 | 521 ± 118 | 0.775 |
eGFR < 60 ml/min/m2, n (%) | 5 (2) | 12 (5) | < 0.001 |
Elevated NT-proBNP, n (%) | 34 (15) | 32 (14) | 0.961 |
Elevated CRP, n (%) | 22 (9) | 28 (12) | 0.289 |
Elevated ESR, n (%) | 83 (36) | 87 (37) | 0.520 |
Elevated CPK, n (%) | 152 (65) | 137 (59) | 0.480 |
ANA-positive, n (%) | 210 (90) | — | — |
Decreased FEV1%, n (%) | 23 (10) | 19 (8) | 0.651 |
Decreased FVC%, n (%) | 40 (17) | 43 (18) | 0.454 |
Decreased DLCO%, n (%) | 159 (68) | 158 (68) | 0.360 |
Decreased VO2max%, n (%) | 53 (23) | 40 (29)* | 0.045 |
Holter ECG abnormalities, n (%) | 81 (35)** | — | — |
Echocardiographic variables | |||
LVEDd, mm, mean ± SD | 47 ± 5 | 47 ± 5 | 0.171 |
LVEDV, ml, mean ± SD | 81 ± 25 | 78 ± 22 | 0.011 |
LVESV, ml, mean ± SD | 31 ± 12 | 31 ± 12 | 0.506 |
LVEF %, mean ± SD | 62 ± 7 | 61 ± 8 | 0.124 |
Left atrial diameter, mm, mean ± SD | 34.6 ± 5.0 | 35.4 ± 4.7 | 0.010 |
E mm/s, mean ± SD | 78 ± 18 | 75 ± 20 | 0.063 |
E/A ratio, mean ± SD | 1.13 ± 0.36 | 1.08 ± 0.39 | 0.009 |
Lateral E′ cm/sec, mean ± SD | 11.19 ± 2.77 | 11.03 ± 3.01 | 0.209 |
Lateral E′ ≤ 10 cm/sec, n (%) | 75 (32) | 108 (46) | 0.002 |
Lateral E′ ≤ 10 cm/sec de novo, n (%) | — | 31 (13) | — |
sPAP mmHg, mean ± SD | 26 ± 8 | 28 ± 11 | 0.019 |
Elevated sPAP, n (%) | 18 (9) | 27 (12) | < 0.001 |
Elevated sPAP de novo, n (%) | — | 13 (6) | — |
TAPSE, mm, mean ± SD | 23 ± 4 | 22 ± 4 | 0.007 |
Pericardial effusion, n (%) | 9 (4) | 17 (7) | < 0.001 |
Pericardial effusion de novo, n (%) | — | 12 (5) | — |
GLS %, mean ± SD*** | −20.91 ± 2.01 | −19.30 ± 2.46 | < 0.001 |
↵* 98 patients with missing data.
↵** 8 patients with missing data.
↵*** Speckle-tracking strain feasible in both baseline and followup echocardiogram in 204 patients. ACEi: angiotensin-converting enzyme inhibitor; ANA: antinuclear antibodies; ARB: angiotensin II receptor blocker; CPK: creatine phosphokinase, cutoff value 145 U/l; CRP: C-reactive protein, cutoff value 10 mg/l; E/A: peak early (E) and late (A) diastolic velocities; ECG: electrocardiogram; eGFR: estimated glomerular filtration rate; ESR: erythrocyte sedimentation rate, cutoff value 20 mm; FEV1: forced expiratory volume in 1 s, percent of predicted value, cutoff value 70%; FVC: forced vital capacity, percent of predicted value, cutoff value 80%; GLS: global longitudinal strain; LV: left ventricular; LVEDd: LV end-diastolic diameter; LVEDV: LV end-diastolic volume; LVESV: LV end-systolic volume; LVEF: LV ejection fraction; mRSS: modified Rodnan skin score; NT-proBNP: N-terminal pro-brain natriuretic peptide, cutoff value 300 ng/l; sPAP: systolic pulmonary arterial pressure; TAPSE: tricuspid annular plane systolic excursion; VO2max: maximum O2 uptake during exercise, percent of predicted value, cutoff value 80%; IQR: interquartile range.