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Table 1 Characteristics of patients with SSc-PAH

From: Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

Characteristic Mean (± SD), number (percent) or median (IQR)
Total number of patients 132
Female 112 (85%)
Age at PAH diagnosis, years 62.3 (± 10.9)
Disease durationa at PAH diagnosis, years 14.1 (± 11.9)
Status at censoring
 Alive 70 (53.0%)
 Dead 60 (45.5%)
 Withdrawn 1 (0.8%)
 Unable to contact 1 (0.8%)
Race
 Caucasian 112 (84.9%)
 Asian 6 (4.6%)
 Aboriginal-Islander 1 (0.8%)
 Hispanic 1 (0.8%)
Follow-up durationb, years (median (IQR)) 3.8 (1.6–5.8)
Survival from PAH diagnosis, years (median (IQR)) 4.0 (2.2–6.2)
Disease durationa at PAH diagnosis, years 14.4 ± 12.1
Disease subtype
 Limited 91 (68.9%)
 Diffuse 30 (22.7%)
 MCTD 7 (5.3%)
Autoantibody status
 Anti-centromere pattern ANA 63 (51.6%)
 Antiphospholipid antibodies (>ULN) 33 (30%)
 RNA polymerase III positive 8 (11.4%)
 Scl 70 positive 9 (7.4%)
WHO functional class at time of PAH diagnosis
 Class I 3 (2.3%)
 Class II 23 (17.4%)
 Class III 79 (59.9%)
 Class IV 12 (9.1%)
Baseline 6MWD, m 326.13 (±105.5)
Baseline mRAP, mmHg 8.3 (± 4.3)
Baseline mPAP, mmHg 35.6 (± 10.4)
Baseline PAWP, mmHg 10.5 (± 3.4)
Baseline mCI, L/min/m2 3.2 (± 1.9)
Baseline PVR, Wood units 8.7 (± 3.8)
Presence of a pericardial effusion at PAH diagnosis 24 (18.2%)
Mean DLCO, % predicted mL/min/mmHg 46.6 (± 13.5)
Mean DLCO/VA, % predicted mL/min/mmHg 56.7 (± 20.2)
Medical therapy
 Pulmonary vasodilator therapyc
  Monotherapy 91 (68.9%)
  Combination therapy 41 (31.1%)
  Warfarin therapyd 37 (28.5%)
  Hydroxychloroquine therapyd 12 (9.1%)
  Antiplatelet agentc 48 (36.9%)
  Mycophenolate mofetil therapyd 7 (5.4%)
  Hormone replacement therapyd 16 (12.3%)
  Proton pump inhibitord 105 (80.8%)
  Home oxygend 28 (21.5%)
  1. Abbreviations: SSc systemic sclerosis, PAH pulmonary arterial hypertension, MCTD mixed connective tissue disease, ANA antinuclear antibody, ULN upper limit of normal, WHO World Health Organization, 6MWD six-minute walk distance, mRAP mean right atrial pressure, mPAP mean pulmonary arterial pressure, PAWP pulmonary artery wedge pressure, PVR peripheral vascular resistance, mCI mean cardiac index, DLCO diffusing capacity of the lung for carbon monoxide, DLCO/VA DLCO adjusted for alveolar volume
  2. aDisease duration from first non-Raynaud manifestation
  3. bFollow-up duration was defined as years from study enrollment
  4. cMonotherapy is treatment with a single PAH-specific therapy. Combination therapy is treatment with more than one specific PAH agent from different classes at one time
  5. dTreatment ever following the diagnosis of PAH