Table 1.

Clinical characteristics and echocardiographic variables at baseline and followup in the total patient population.

N = 234BaselineFollowupp
Age, mean ± SD52 ± 14
Female, n (%)196 (84)
Cardiovascular medication, n (%)
  ACEi/ARB135 (38)
  Beta-blocker29 (8)
  Ca2+ channel-blocker155 (44)
  Diuretics49 (14)
Immunosuppressive medication, n (%)
  Corticosteroids30 (9)
  Cyclophosphamide4 (1)
  Methotrexate32 (9)
  Azathioprine12 (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 ± SD513 ± 128521 ± 1180.775
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 ± SD47 ± 547 ± 50.171
  LVEDV, ml, mean ± SD81 ± 2578 ± 220.011
  LVESV, ml, mean ± SD31 ± 1231 ± 120.506
  LVEF %, mean ± SD62 ± 761 ± 80.124
  Left atrial diameter, mm, mean ± SD34.6 ± 5.035.4 ± 4.70.010
  E mm/s, mean ± SD78 ± 1875 ± 200.063
  E/A ratio, mean ± SD1.13 ± 0.361.08 ± 0.390.009
  Lateral E′ cm/sec, mean ± SD11.19 ± 2.7711.03 ± 3.010.209
  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 ± SD26 ± 828 ± 110.019
  Elevated sPAP, n (%)18 (9)27 (12)< 0.001
  Elevated sPAP de novo, n (%)13 (6)
  TAPSE, mm, mean ± SD23 ± 422 ± 40.007
  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.