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Juvenile Scleroderma-What has Changed in the Meantime?
Amra Adrovic1, Sezgin Sahin1, Kenan Barut1
1Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
Current Rheumatology Reviews
|April 26, 2018
Summary
Juvenile scleroderma, a rare connective tissue disease, presents systemic and localized forms. Current treatments combine disease-modifying anti-rheumatic drugs and steroids, with biologics showing promise for severe cases.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Connective Tissue Diseases
Background:
- Juvenile scleroderma is a rare chronic connective tissue disorder affecting children, characterized by skin stiffening.
- The disease occurs in approximately one in a million children and is classified into systemic and localized forms based on organ involvement.
- Systemic juvenile scleroderma involves skin, musculoskeletal, and vascular systems, with rare cardiovascular, gastrointestinal, and renal complications.
Purpose of the Study:
- To review recent findings and new developments in juvenile scleroderma due to its rarity and limited research.
- To provide an updated overview of systemic and localized juvenile scleroderma, including clinical features and therapeutic strategies.
Main Methods:
- A systematic literature search was conducted using PubMed/Medline and the Cochrane Library.
- The search focused on recent randomized controlled trials, case-control studies, cohort studies, and case reports concerning juvenile scleroderma.
- Both systemic and localized forms of the disease were included in the review.
Main Results:
- Systemic juvenile scleroderma primarily manifests with skin, musculoskeletal, and vascular issues; internal organ involvement is infrequent in children.
- First-line therapy for systemic juvenile scleroderma involves a combination of disease-modifying anti-rheumatic drugs (e.g., methotrexate, mycophenolate mofetil, cyclosporine) and steroids.
- Bosentan is utilized for pulmonary hypertension and digital ulcerations, while biological treatments offer options for severe disease; localized scleroderma lacks specific therapies.
Conclusions:
- Current understanding of juvenile scleroderma is often extrapolated from adult studies, highlighting a need for pediatric-specific research.
- Multicenter, prospective studies are crucial for clarifying the clinical course and prognosis of juvenile scleroderma.
- Genetic factors, particularly HLA class II, are implicated in pathogenesis, and emerging biological agents show therapeutic potential.
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