Table 2 Repartition between the diffuse and limited cutaneous subset of the disease in our cohort and specific data concerning overlap syndromes (including patients with diffuse and limited jSSc). Results are presented into absolute values, and as a percentage of the subgroup in parentheses. The values and proportions presented in this table refer to the entire patient cohort unless otherwise stated. Data are compared using the Chi-square test (p < 0,05). ANA, antinuclear antibodies; CO, carbon monoxide; GI, gastro-intestinal; jSSc, juvenile systemic scleroderma; mRSS, modified Rodnan skin score; SD, standard deviation

Age ≤ 10 (n = 14)

3

9

0,019*

Age > 10 (n = 6)

5

1

Mean mRSS (+/- SD)

n = 7

16.4 (+/- 6.18)

n = 6

5.5 (+/- 6.92)

n = 4

5

Articular involvement

4 (50)

7 (70)

2 (40)

Muscular involvement

1 (13)

3 (30)

2 (40)

GI involvement

7 (87)

7 (70)

3 (60)

Pulmonary involvement

7 (87)

7 (70)

4 (80)

 Restrictive lung disease

6 (75)

3 (30)

3 (60)

 Interstitial lung disease

4 (50)

3 (30)

2 (40)

 Low CO transfer

6 (75)

4 (40)

 Pulmonary hypertension

1 (13)

0

0

Cardiac involvement

5 (62)

1 (10)

2 (40)

Growth retardation

n = 5

4 (80)

n = 9

3 (33)

n = 5

3 (60)

Antibodies

 ANA

8

10

5 (100)

 Anti-Scl70

n = 7

5 (71)

3 (30)

2 (40)

 Anti-centromeres

n = 7

0

2 (20)

0

 Anti-PmScl

n = 7

0

2 (20)

2 (40)

 Anti-fibrillarin

n = 7

1 (14)

1 (10)

0

 Anti-Ku

n = 7

0

1 (10)

1 (20)

Treatments used

 Corticosteroids

8 (100)

6 (75)

5 (100)

 Methotrexate

4 (50)

6 (75)

4 (80)

 Mycophenolate

6 (75)

2 (25)

2 (40)

 Cyclophosphamide

1 (13)

0

0

 Rituximab

2 (25)

0

0

 Tocilizumab

2 (25)

0

0