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Abstract Number: 2178

Juvenile Onset Systemic Sclerosis: Clinical and Serological Features, and Mortality In Comparison With Adult Onset Disease

Juan G. Ovalles-Bonilla1, Francisco Javier López-Longo2, Indalecio Monteagudo1, Esperanza Naredo1, Carlos Gonzalez Fernandez1, María Montoro Alvarez1, Lina Martínez-Estupiñán1, Juan C. Nieto3, Julia Martínez-Barrio1, Michelle Hinojosa1, Natalia Bello1, Belen Serrano1, Carmen Mata1 and Luis Carreño1, 1Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain, 2Hospital General Universitario Gregorio Marañón, Department of Rheumatology, Madrid, Spain, 3Hospital General Universitario Gregorio Marañón, Madrid, Spain

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Juvenile scleroderma, Morbidity and mortality and systemic sclerosis

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Session Information

Title: Pediatric Rheumatology-Clinical and Therapeutic Aspects III: Juvenile Idiopathic Arthritis and Other Pediatric Rheumatic Diseases

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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