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Treatment in Juvenile Scleroderma

  • Pediatric Rheumatology (S Ozen, Section Editor)
  • Published:
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Abstract

Purpose of Review

Treatment of scleroderma in children is challenging since little is known about its pathogenesis. Herein, we review the most recent evidence regarding the treatment of juvenile scleroderma.

Recent Findings

According to the recent recommendations for Pediatric Rheumatology in Europe (SHARE), systemic treatment in localized scleroderma is needed when there is a risk for disability, such as in generalized or pansclerotic morphea and progressive linear scleroderma. In juvenile systemic sclerosis, the introduction of the severity score, J4S, has standardized the assessment of the patients in the daily practice and allowed a more tailored therapeutic approach. Since, to date, no clinical trial is available in JSSc, due to its rarity, the treatment is based on adults’ experience.

Summary

The recent recommendations for juvenile scleroderma represent an important instrument to standardize the treatment approach, confirm the role of methotrexate, and open new windows for effective experimental treatments, such as mycophenolate mofetil and biological agents, for severe or refractory cases.

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Notes

  1. Martini G, Saggioro L, Culpo R, Vittadello F, Meneghel A, Zulian F. Mycophenolate mofetil for methotrexate-resistant Juvenile Localised Scleroderma. Rheumatology (Oxford) 2020 (submitted).

  2. Foeldvari I, Culpo R, Sperotto F, et al. Consensus-based recommendations for the management of Juvenile Systemic Sclerosis. Rheumatology (Oxford) 2020 (submitted).

  3. Khanna D, Lin CJF, Kuwana M, et al. Efficacy and safety of tocilizumab for the treatment of systemic sclerosis: results from a phase 3 randomized controlled trial [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10).

  4. Bhattacharyya S, Wang W, Wie J, et al. Pharmacological inhibition of JAK/STAT signaling by tofacitinib prevents experimental organ fibrosis: novel therapy for systemic sclerosis [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10).

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Zulian, F., Tirelli, F. Treatment in Juvenile Scleroderma. Curr Rheumatol Rep 22, 45 (2020). https://doi.org/10.1007/s11926-020-00910-x

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