ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1326

Transition of Care and Long-Term Outcomes of Juvenile Systemic Sclerosis during Adulthood: Results from a French Single-Center Case-Control Study

Francois-Xavier Mauvais1, Brigitte Bader-Meunier2, Alice Berezne3, Guillaume Bussone4, Christine Bodemer5, Loïc Guillevin6, Pierre Quartier2 and Luc Mouthon6, 1INSERM U1151 / CNRS 8253, PARIS, France, 2Pediatric Rheumatology, IMAGINE Institute, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris, Université Paris-Descartes, Paris, France, 3Paris Descartes University, Internal Medicine department, Cochin Hospital, Paris, France, 4Internal Medicine, Hopital Cochin, Paris, France, 5Hôpital Necker-Enfants Malades, Paris, France, 6National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: ,

Session Information

Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects: Pediatric Lupus, Scleroderma and Myositis (ACR)

Session Type: Abstract Submissions (ACR)

Background/Purpose

To describe the transition of care from Pediatric to an adult Internal Medicine department and long-term outcomes of patients with juvenile-onset SSc.

Methods

Twenty patients with SSc diagnosed before the age of 17 and previously followed in Pediatric departments were included. The control group comprised 60 patients randomly selected from all patients with a SSc diagnosed after 18 years old, matching by sex and disease duration.

Results

In the juvenile-onset group, 16/20 (80%) patients were female and 11/20 (55%) presented a diffuse SSc. Mean age for diagnosis was 11.9 years (5.1-15.9). One patient had anti-centromere, 4/20 (20%) anti-Scl70 and 1/20 (5%) anti-U1RNP autoantibodies.

While juvenile-onset patients had a significantly higher incidence of calcinosis (9/20 (45%) vs 12/60 (20%); p=0.04), they had a lower incidence of interstitial lung disease (4/20 (20%) vs 32/60 (53.3%); p=0.01) compared to the adult-onset group.

At the time of last follow-up, mean disease duration was 14.2±13.1 years. Survival rate was lower among the juvenile-onset group but this difference was not significant (17/20 (85%) vs 57/60 (95%); p=0.16). Bowel involvement had a significantly higher incidence (11/20 (55%) vs 6/60 (10%); p<0.001) in the juvenile-onset group as well as calcinosis (13/20 (65%) vs 15/20 (25%); p<0.02) compared to the adult-onset group. Juvenile-onset patients had received significantly more steroids > 15mg/d (11/20 (55%) vs 7/60 (11.7%); p<0.001) and methotrexate (10/20 (50%) vs 8/60 (13.3%); p=0.002). No significant difference could be observed regarding biological parameters between the two groups.

Only one patient in the juvenile-onset group had a major impact on quality of life, with a significant delay in his education and a depressive syndrome. Factors associated with a poor prognosis in juvenile-onset SSc were lung fibrosis and pericarditis.

Conclusion

At the time of transition of care to adult structures, patients with juvenile-onset SSc present with important musculoskeletal damages and lower incidence of lung involvement than patients with adult-onset SSc.

Disclosure:

F. X. Mauvais,
None;

B. Bader-Meunier,
None;

A. Berezne,
None;

G. Bussone,
None;

C. Bodemer,
None;

L. Guillevin,
None;

P. Quartier,
None;

L. Mouthon,
None.

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