Research Article

Exhaled Nitric Oxide in Systemic Sclerosis Lung Disease

Table 1

Summary of subject characteristics.

SSc SSc-PAH SLE-PAH SSc-ILD limited SSc-ILD extensive IPAH Control

Female sex (%)16 (100%)5 (71%)7 (100%)7 (88%)3 (75%)7 (78%)5 (50%)
Age in years (median)51513754.556.541.033.5
Limited cutaneous subtype (%)15 (94%)5 (71%)NA7 (88%)2 (50%)NANA
Manifestations (%)
Calcinosis6 (38%)2 (29%)NA1 (13%)2 (50%)NANA
Raynaud’s phenomenon16 (100%)7 (100%)NA8 (100%)4 (100%)NANA
Esophageal dysmotility13 (81%)6 (86%)NA5 (63%)4 (100%)NANA
Sclerodactyly12 (75%)6 (86%)NA5 (63%)4 (100%)NANA
Telangiectasia13 (81%)7 (100%)NA5 (63%)3 (75%)NANA
Renal crisis00NA00NANA
Abnormal nailfold capillaries5 (31%)5 (71%)NA3 (38%)2 (50%)NANA
Digital ulcers3 (19%)5 (71%)NA2 (25%)2 (50%)NANA
ScL-70 antibody1 (6%)0NA2 (25%)2 (50%)NANA
Anti-centromere antibody6 (38%)1 (14%)NA2 (25%)1 (25%)NANA
Hemodynamics
mPAP mmHg (median, IQR)NA40 (37–48)42 (39–48)NA38 (34–40)44 (42–53)NA
LVEDP mmHg (median, IQR)NA8 (4–10)6 (6–11)NA6 (4–8)9 (7–13)NA
Comorbidities (%)
Asthma2 (13%)02 (29%)1 (13%)02 (22%)0
COPD0000000
OSA2 (13%)01 (14%)1 (13%)01 (11%)0
Systemic hypertension4 (25%)1 (14%)1 (14%)2 (25%)2 (50%)3 (33%)0
Atrial fibrillation00001 (25%)00
CAD1 (6%)1 (14%)0001 (11%)0
Smoking history (%)
Current2 (13%)1 (14%)0000NA
Former6 (38%)3 (43%)2 (29%)2 (25%)2 (50%)3 (33%)NA
Medication (%)
NSAID2 (13%)2 (29%)2 (29%)5 (63%)02 (22%)0
Prednisone02 (29%)5 (71%)3 (38%)2 (50%)00
Inhaled corticosteroids1 (6%)001 (13%)01 (11%)0
Pulmonary function tests
FEV1% predicted (median)92.095.078.085.071.087.5NA
FVC% predicted (median)97.095.081.089.065.090.5NA
TLC% predicted (median)9610483957490NA
DLCO% predicted (median)72.048.573.558.065.570.5NA

mPAP: mean pulmonary artery pressure, LVEDP: left ventricular end-diastolic pressure, COPD: chronic obstructive pulmonary disease, OSA: obstructive sleep apnea, CAD: coronary artery disease, NSAID: nonsteroidal anti-inflammatories, FEV1: forced expiratory volume in one second, FVC: forced vital capacity, TLC: total lung capacity, DLCO: diffusing capacity.