Table 1

Baseline characteristics

Patients, number

21

Gender male/female

1

/

20

Age, years

52

±

18

Height, cm

165

±

6

Weight, kg

68

±

11

WHO functional class, number (%)

II

9

(43)

III

7

(33)

IV

5

(24)

Lung function

TLCO, %

55

±

18

FEV1%VC, %

78

±

7

pO2, mmHg

69

±

12

Mean 6-minute-walking distance, meter

386

±

121

Differential Diagnosis, number (%)

Systemic sclerosis

8

(43%)

Systemic lupus

7

(33%)

MCTD

2

(10%)

Others

4

(14%)

Cardiac catherization

Pulmonary artery pressure, mmHg

49

±

13

Pulmonary vascular resistance, dyn×sec×cm-5

789

±

498

Right atrium pressure, mmHg

7

±

3

Pulmonary artery oxygen saturation, %

62

±

10

PCWP, mmHg

10

±

5

Cardiac index, L×min×m-2

2.6

±

0.6

PAH-targeted medication, number (%)

Endothelin receptor antagonists

12

(57%)

Phosphodiesterase-5-Inhibitors

15

(71%)

Prostanoids inhaled

3

(14%)

Prostanoids intravenous

1

(5%)

Calcium channel blockers

2

(10%)

Soluble guanyl cyclase-stimulator

3

(14%)

Combination therapy, number (%)

Monotherapy

8

(38%)

Dual therapy

10

(48%)

Triple therapy

3

(14%)


Results are presented as mean ± standard deviation unless stated otherwise

WHO: World Health Organization; FEV1%VC: forced expiratory volume in one second/vital capacity; TLCO: carbon monoxide transfer factor; pO2: partial oxygen pressure; PCWP: pulmonary capillary wedge pressure; MCTD: mixed connective tissue disease.

Grünig et al. Arthritis Research & Therapy 2012 14:R148   doi:10.1186/ar3883

Open Data