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

Morrisroe, Kathleen; Stevens, Wendy; Huq, Molla; Prior, David; Sahhar, Jo; Ngian, Gene-Siew; Celermajer, David; Zochling, Jane; Proudman, Susanna; Nikpour, Mandana

doi:10.1186/s13075-017-1341-x

Arthritis Research & Therapy

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 duration^a 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Withdrawn	1 (0.8%)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 duration^b, years (median (IQR))	3.8 (1.6–5.8)
Survival from PAH diagnosis, years (median (IQR))	4.0 (2.2–6.2)
Disease duration^a 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 therapy^c
Monotherapy	91 (68.9%)
Combination therapy	41 (31.1%)
Warfarin therapy^d	37 (28.5%)
Hydroxychloroquine therapy^d	12 (9.1%)
Antiplatelet agent^c	48 (36.9%)
Mycophenolate mofetil therapy^d	7 (5.4%)
Hormone replacement therapy^d	16 (12.3%)
Proton pump inhibitor^d	105 (80.8%)
Home oxygen^d	28 (21.5%)

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
^aDisease duration from first non-Raynaud manifestation
^bFollow-up duration was defined as years from study enrollment
^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
^dTreatment ever following the diagnosis of PAH

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