Limited cutaneous systemic sclerosis skin demonstrates distinct molecular subsets separated by a cardiovascular development gene expression signature

Table 2 Clinical and laboratory parameters of the limited-1 and limited-2 subgroups

Clinical feature		Limited 1 (n = 10)	Limited 2 (n = 5)
MRSS, median (range)		5.5 (3–10)	6 (5 − 7)
Disease duration from first non-Raynaud’s symptom, median (range) years		16 (4–40)	6 (2–28)
ANA primary pattern, N (%) patients	Homogenous	3 (30)	1 (20)
	Speckled	1 (10)	1 (20)
	Centromere	5 (50)	1 (20)
	Nucleolar	0	1 (20)
SSc-specific antibodies	Scl-70	3 (30)	1 (10)
SSc-specific antibodies	RNA pol III	0	0
Vasculopathy, N (%) patients	Digital ulcers	4 (40)	2 (40)
	PAH	0	0
	Renal crisis	0	0
Interstitial lung disease, N (%)		2 (20)	2 (40)
Inflammatory arthritis, N (%)		3 (30)	0
Vascular therapies, N (%) patients	ARB	8 (80)	2 (40)
	Ca channel antagonist	2 (20)	2 (40)
	Iloprost	3 (30)	1 (20)
	PDE5 inhibitor	1 (10)	2 (40)
Immunosuppressive therapies, N (%) patients	PDN	2 (20)	2 (40)
	MMF	2 (20)	1 (20)
	MTX	2 (20)	0
	AZA	1 (10)	0
	HCQ	2 (20)	1 (20)

ANA antinuclear antibodies, SSc systemic sclerosis, MRSS modified Rodnan skin score, PAH pulmonary arterial hypertension, ARB angiotensin receptor blocker, PDE cGMP-regulated phosphodiesterase, MTX methotrexate, HCQ hydroxychloroquine, PDN prednisolone, AZA azathioprine