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Juvenile Scleroderma: A Referral Center Experience.

Amra Adrovic1, Sezgin Şahin1, Kenan Barut1

  • 1Department of Pediatric Rheumatology, İstanbul University, Cerrahpasa Medical School, İstanbul, Turkey.

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Summary
This summary is machine-generated.

Juvenile systemic sclerosis (JSS) and juvenile localized scleroderma (JLS) require close monitoring for internal organ involvement to prevent complications. While rare, vital organ impact significantly affects prognosis, with biological agents offering an alternative for treatment-resistant cases.

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Juvenile localized sclerodermaRaynaud's phenomenonjuvenile systemic sclerosisskin stiffening

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Area of Science:

  • Pediatric Rheumatology
  • Dermatology
  • Immunology

Background:

  • Juvenile systemic sclerosis (JSS) and juvenile localized scleroderma (JLS) are rare autoimmune conditions affecting children.
  • Understanding the specific features and outcomes of these conditions is crucial for effective management.

Purpose of the Study:

  • To evaluate the demographic and clinical characteristics of JSS and JLS patients.
  • To analyze laboratory data, treatment modalities, and outcomes in a Turkish pediatric rheumatology center.

Main Methods:

  • Retrospective longitudinal study of 57 patients (29 JSS, 28 JLS) diagnosed between 2006 and 2015.
  • Analysis of medical records for demographic, clinical, laboratory findings, and treatment data.
  • Minimum six-month follow-up period for all included patients.

Main Results:

  • Mean age at onset was 9.9 years for JSS and 7.7 years for JLS.
  • Interstitial lung disease (27%) and pulmonary hypertension (10%) were noted in JSS.
  • Corticosteroids with methotrexate were common; biological agents were used for refractory cases.

Conclusions:

  • Close monitoring for internal organ involvement is vital in JSS and JLS to prevent severe complications.
  • Vital organ involvement, though rare, has a profound impact on prognosis.
  • Biological agents are a viable treatment option for patients unresponsive to standard immunosuppressive therapies.