Systemic sclerosis sera affect fibrillin-1 deposition by dermal blood microvascular endothelial cells: therapeutic implications of cyclophosphamide

Table 1 Demographic and clinical characteristics of the 21 patients with systemic sclerosis^a

Characteristics	SSc patients (N= 21)
Mean age (±SD), yr	56.5 ± 12.1
Sex, n (%)
Male	4 (19.1)
Female	17 (80.9)
Disease subset, n (%)
lSSc	13 (61.9)
dSSc	8 (38.1)
Mean disease duration (±SD), yr^b	7.7 ± 4.1
Autoantibody positivity, n (%)
ANA	21 (100)
Anti-Scl-70	7 (33.3)
ACA	10 (47.6)
Digital ulcers, n (%)	12 (57.1)
Nailfold capillaroscopy pattern, n (%)
Early	4 (19.0)
Active	8 (38.1)
Late	9 (42.9)
Mean skin score (±SD)	10.2 ± 6.3
ILD, n (%)^c	11 (52.4)
CYC treatment, n (%)	13 (61.9)^d

^aACA, anticentromere antibodies; ANA, antinuclear antibodies; Anti-Scl-70, anti-Scl-70 antibodies; CYC, cyclophosphamide; dSSc, diffuse cutaneous systemic sclerosis; ILD, interstitial lung disease; lSSc, limited cutaneous systemic sclerosis; SSc, systemic sclerosis. Except where indicated otherwise, values are the number (%) of participants. ^bDisease duration was calculated from the date of the first non-Raynaud's symptom of SSc. ^cDetermined by high-resolution computed tomography scan. ^dEight patients with lSSc and five patients with dSSc.

ISSN: 1478-6362