Changes in plasma CXCL4 levels are associated with improvements in lung function in patients receiving immunosuppressive therapy for systemic sclerosis-related interstitial lung disease

Table 1 Baseline characteristics of SLS II participants with CXCL4 measurements at baseline

	Cyclophosphamide		Mycophenolate
Characteristic	N		N
Age (years)	71	52.3 ± 9.5	65	52.6 ± 10.0
Female sex (% of patients)	71	77.5	65	69.2
Duration of scleroderma (years)	70	2.5 ± 1.8	63	2.7 ± 1.7
% Limited/% diffuse	71	45.1/54.9	65	36.9/63.1
FVC (%-predicted)	71	66.2 ± 9.9	65	66.6 ± 8.2
FEV1:FVC (%-predicted)	71	83.5 ± 5.6	65	81.8 ± 5.7
TLC (%-predicted)	71	65.4 ± 12.1	65	66.5 ± 10.2
DLCO (%-predicted)^a	71	53.8 ± 14.2	65	54.4 ± 11.3
DL/VA (%-predicted)	71	61.0 ± 13.7	65	60.9 ± 11.8
Mahler Dyspnea Index (focal score)	67	7.0 ± 2.3	61	7.3 ± 2.1
Skin-thickening score (mRSS)^b
All patients	71		65
Mean		14.1 ± 10.8		15.2 ± 10.1
Range		2–46		1–41
Patients with dcSSc	39		41
Mean		20.7 ± 9.9		20.7 ± 8.6
Range		3–46		4–41
Patients with lcSSc	32		24
Mean		6.1 ± 4.3		5.9 ± 3.4
Range		2–18		1–14
HAQ disability index (0–3)^c	71	0.7 ± 0.7	65	0.7 ± 0.6
QLF-WL	69	9.1 ± 7.0	62	8.2 ± 6.9
QLF-LM	69	23.2 ± 19.2	62	22.5 ± 19.9
QILD-WL	69	32.1 ± 14.2	62	27.2 ± 13.6
QILD-LM	69	53.2 ± 19.3	62	49.8 ± 21.0
Auto-antibody (% positive in patients tested)
ANA	70	94.3	62	96.8
Topoisomerase-1	70	45.7	62	46.2
RNA polymerase III	70	11.4	62	14.5
Centromere	70	2.9	62	1.6
Th\to	67	7.5	60	5.0

Values are mean ± standard deviation, unless otherwise noted
^aAdjusted for hemoglobin
^bScores for skin thickening (mRSS) can range from 0 to 51, with higher scores indicating more severe thickening
^cScores for HAQ Disability Index can range from 1 to 3, with higher numbers indicating greater disability
FVC forced vital capacity, FEV ₁ forced expired volume in 1 second, TLC total lung capacity, DLCO diffusing capacity of the lung for carbon monoxide, DL/VA ratio of DLCO to alveolar volume, mRSS modified Rodnan skin score, dcScc diffuse cutaneous systemic sclerosis, lcSSc limited or cutaneous systemic sclerosis, HAQ Health Assessment Questionnaire, QLF quantitative extent of lung fibrosis on high-resolution chest computed tomography, QILD quantitative extent of total interstitial lung disease (including fibrosis, honeycomb, and ground glass opacity), WL whole lung, LM lobe of maximal involvement
ANA Anti-nuclear antibody, Th/To Th/To ribonucleoprotein antibody

ISSN: 1478-6362