Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: Currently data regarding long-term outcome of juvenile systemic sclerosis (jSSc) is scarce. To describe the differences between patients with jSSc versus adult onset evaluated at a single medical center.
Methods: Patients with disease onset before the age of 17 years were included in the jSSc group, while subjects with SSc onset after age 18 formed the adult-onset cohort. The 2 groups were compared with respect to disease classification, clinical, serological data, and mortality.
Results: Seventeen jSSc cases seen between 1986 and 2011 were compared with 120 adult onset SSc cases. Mean age at onset in the jSSc group was 11.7±3.9 years, and the mean disease duration at the last follow-up was 19.3±7.8 years. The sex distribution was 7.5:1 (female:male). Among juvenile cases, 58.8% had diffuse SSc, while 41.2% had limited SSc. The clinical features seen in juvenile patients were: articular manifestations (arthralgia, arthritis, contractures and weakness) in the 100% of cases, Raynaud’s phenomenon in 94%, digital ulcers and telangiectasia in 53%, interstitial lung disease in 11.8%. Antinuclear antibody was positive in 100%, and anti-U1RNP in 64.7%. A global mortality of 17.6% was seen. Articular manifestations, anti-DNA and anti-U1RNP were seen more frequently in the jSSc group. Adult onset SSc develop a higher frequency of sclerodactyly and interstitial lung disease.
Conclusion: Diffuse SSc, limited SSc, calcinosis, digital ulcers, pulmonary hypertension, anti-centromere, anti-Scl-70 and mortality shows a similar distribution between the 2 groups. Patients with jSSc presented a lower frequency of interstitial lung disease and sclerodactyly, but a higher expression of anti-DNA and anti-U1RNP.
|
Table 1: Clinical and serologic features, and mortality in comparison with adult onset disease |
|||
|
FEATURES (%) |
Juvenile SSc n=17 |
Adult SSc n=120 |
p |
|
Age at disease onset ± DE |
11.7±3.9 |
46.5±14.4 |
<0.001 |
|
Disease duration ± DE |
19.3±7.8 |
14.6±10.5 |
0.07 |
|
Diffuse SSc |
10 (58.8) |
52 (43.3) |
0.23 |
|
Limited SSc |
7 (41.2) |
68 (56.7) |
|
|
sclerodactyly |
5 (29.4) |
68 (56.7) |
0.03 |
|
Telangiectasia |
9 (52.9) |
74 (61.7) |
0.49 |
|
Digital ulcers |
9 (52.9) |
80 (66.7) |
0.27 |
|
Raynaud’s phenomenon |
16 (94.1) |
111 (92.5) |
0.81 |
|
Pulmonary Hypertension |
2 (11.8) |
9 (7.5) |
0.54 |
|
Interstitial lung disease |
2 (11.8) |
49 (40.8) |
0.02 |
|
Articular manifestations |
17 (100) |
71 (67.5) |
0.005 |
|
Renal manifestations |
3 (17.6) |
17 (14.2) |
0.70 |
|
Anti-centromere |
3 (17.6) |
36 (30) |
0.29 |
|
Anti-Scl-70 |
2 (11.8) |
30 (26.1) |
0.19 |
|
ANA |
17 (100) |
109 (90.8) |
0.19 |
|
Anti-DNA |
5 (31.2) |
11 (9.9) |
0.016 |
|
Anti-U1RNP |
11 (64.7) |
13 (12.9) |
<0.001 |
|
Mortality |
3 (17.6) |
31 (25.8) |
0.46 |
jSSc: Juvenile Systemic Sclerosis, SSc: Systemic Sclerosis, ANA: antinuclear antibodies
Disclosure:
J. G. Ovalles-Bonilla,
None;
F. J. López-Longo,
None;
I. Monteagudo,
None;
E. Naredo,
None;
C. Gonzalez Fernandez,
None;
M. Montoro Alvarez,
None;
L. Martínez-Estupiñán,
None;
J. C. Nieto,
None;
J. Martínez-Barrio,
None;
M. Hinojosa,
None;
N. Bello,
None;
B. Serrano,
None;
C. Mata,
None;
L. Carreño,
None.
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