Research Article

The Role of Pulmonary Function Test for Pulmonary Arterial Hypertension in Patients with Connective Tissue Disease

Table 1

Baseline clinical characteristics of patients with CTD-PAH.

CharacteristicsValue

Total number31
 SLE22 (76.3%)
 SSc9 (23.7%)
Age (years)
Female30 (96.8%)
NT-proBNP (ng/L)
Stabilization of CTD21 (67.7%)
sPAP (mmHg)
RAA (cm2)
RVBD (cm)
RAP (mmHg)
TAPSE (mm)
6MWD (m)
WHO FC
 I6 (19.4%)
 II13 (41.9%)
 III9 (29.0%)
 IV3 (9.7%)
Risk stratification
 Low risk8 (25.8%)
 Intermediate risk15 (48.4%)
 High risk8 (25.8%)
FVC (%)
FEV1/FVC (%)
MEF50 (%)
TLC (%)
DLCO (%)
FVC/DLCO

SLE: systemic lupus erythematosus; SSc: systemic sclerosis; CTD: connective tissue disease; NT-proBNP: N-terminal prohormone of brain natriuretic peptide; sPAP: systolic pulmonary arterial pressure; RAA: right atrial area; RVBD: right ventricular basal diameter; RAP: right atrial pressure; TAPSE: tricuspid annular plane systolic excursion; 6MWD: 6-minute walking distance; WHO FC: World Health Organization functional class; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 second; MEF50: maximum expiratory flow at 50% of vital capacity; TLC: total lung capacity; DLCO: diffusing capacity of the lung for carbon monoxide.