Juvenile Scleroderma-What has Changed in the Meantime?

Background: Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was reported as one per million. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. Objective: Since it is uncommon in children, many aspects of the disease remain discussable. With this review, we aimed to revise recent findings and new developments in this rare condition. Method: A systematic literature research was performed, using the following medical databases: Pubmed/ Medline and the Cochrane Library. We searched for up-to-date randomized controlled studies, case-control studies, and cohort studies and cases reports on juvenile scleroderma (both systemic and localized form). Results: Skin manifestations are most prominent features of the systemic form, followed by musculoskeletal and vascular involvement. Cardiovascular, gastrointestinal and renal disorders are rare in childhood. Combination of disease modifying anti- rheumatic drugs (methotrexate, mycophenolatemofetil, cyclosporine) and steroid reprents the first line therapy. Bosentan is used for cases with pulmonary hypertension and for extensive digital ulcerations. Biological treatment emerges as a useful treatment option in most severe form of the disease. Localized scleroderma is characterized with sclerodermatosis of the skin. Internal organ involvement is not expected. Classification of the local scleroderma is made according to the size and localization of the skin changes. There are few different therapeutical options but there is no specific therapy for the localized scleroderma. Conclusion: Many data regarding disease features and treatment options in juvenile scleroderma are based on studies among adults. There is a striking need for multicentric, prospective studies among children with juvenile scleroderma, in order to elucidate some questions of clinical course and disease prognosis. Recent genetic studies have revealed the role of the genetic factors (namely HLA class II) in the pathogenesis of the disease. Emerging biological agents and new treatment options are showing promising results.