- Poster presentation
- Open Access
Juvenile systemic sclerosis: review of 15 patients
© Sousa et al; licensee BioMed Central Ltd. 2014
- Published: 17 September 2014
- Autologous Bone Marrow
- Pulmonary Involvement
- Autologous Bone Marrow Transplant
- Skin Fibrosis
Introduction: Systemic sclerosis, a rare disease in childhood, is characterized by skin fibrosis, internal organ involvement, and vasculopathy. Juvenile systemic sclerosis (JSSc) represents less than 10% of all scleroderma patients.
Objectives: To describe the clinical characteristics and disease progression of children with JSSc followed in Portuguese pediatric rheumatology centers.
Methods: Clinical and laboratory features as well as medication and outcome of children who met classification criteria for JSSc were reviewed.
Cumulative manifestations and complications observed during the follow-up
Diffuse cutaneous disease
Interstitial lung disease
All but one child were ANA positive (93%), 7 tested positive for anti-Scl70, 2 positive for anti-RNP and 1 for anti-fibrillarin antibodies. There were no cases of anti-centromere antibodies.
Immunosuppressants (93%), proton pump inhibitors (80%), calcium channel blockers (53%) and corticosteroids (60%) were the most common therapeutic options. Five and four children were treated with prostacyclin analogues and ET-1 receptor antagonist, respectively. One child needed autologous bone marrow transplant due to severe refractory disease.
An improvement of skin thickening and stabilization of pulmonary involvement was documented in most cases. No deaths were registered in this cohort. Table 1.
Conclusions: Diffuse cutaneous disease was the subtype of JSSc more prevalent identified in pediatric rheumatology centers. Raynaud's phenomenon as well as capillaroscopic abnormalities are almost universal at disease presentation. Internal organ involvement is common and occurs early during disease course, although clinically silent in several cases.
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