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

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

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)

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)

  1. 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