Table 1.

Study population (n=278)

Age, mean (±SD), yrs	46.9 (13.6)
Gender, female, n (%)	237 (85.3)
Cutaneous involvement, diffuse, n (%)	171 (61.5)
Disease duration, mean (±SD), yrs	6.0 (6.5)
MRSS, mean (±SD)	17.3 (12.3)
Deceased patients, n (%)	83 (29.7)
Survival time (from time of enrollment), mean (±SD), yrs	5.8 (5.0)
Autoantibody profile, %
ANA*	93.1
ACA**	6.2
ATA†	21.8
ANoA‡	44.5
AFAβ	18.5
Pol IIIγ	15.4
U1-RNPΔ	12.2
PM/Scl§	3.2
RoΦ	9.3

^*ANA, anti-nuclear antibodies;

^**ACA, anti-centromere antibodies;

^†ATA, anti-topoisomerase I antibodies;

^‡ANoA, anti-nucleolar antibodies;

^βAFA, anti-fibrillarin antibodies;

^γPol III, RNA polymerase III;

^ΔU1-RNP, U1-ribonucleoprotein;

^§PM/Scl, polymyositis-scleroderma antigen;

^ΦRo, Ro-SS-A/60.