Development of minimum standards of care for juvenile localized scleroderma

T. Constantin, Ivan Foeldvari, Clare E. Pain, Annamária Pálinkás, Peter Höger, Monika Moll, Dana Nemkova, Lisa Weibel, Melinda Laczkovszki, Philip Clements, Kathryn S. Torok

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Juvenile localized scleroderma (jLS), also known as morphea, is an orphan disease. Pediatric guidelines regarding diagnosis, assessment, and management are lacking. Our objective was to develop minimum standards of care for diagnosis, assessment, and management of jLS. A systematic review was undertaken to establish the pediatric evidence for assessment and monitoring of jLS. An expert panel, including members of the Pediatric Rheumatology European Society (PRES) Scleroderma Working Group, were invited to a consensus meeting where recommendations were developed based on evidence graded by the systematic review and, where evidence was lacking, consensus opinion. A nominal technique was used where 75% consensus was taken as agreement. Recommendations for diagnosis, assessment, and management were developed. Due to a lack of pediatric evidence, these were primarily consensus driven. Careful assessment for extra-cutaneous manifestations including synovitis, brain involvement, and uveitis were key features together with joint assessments between Dermatology and Rheumatology to improve and standardize care. Conclusion: Management of jLS is varied. These recommendations should help provide standardization of assessment and care for those with this rare and potentially debilitating condition.(Table presented.)

Original languageEnglish
Pages (from-to)1-17
Number of pages17
JournalEuropean Journal of Pediatrics
DOIs
Publication statusAccepted/In press - May 4 2018

Fingerprint

Localized Scleroderma
Standard of Care
Pediatrics
Rheumatology
Skin Manifestations
Synovitis
Uveitis
Rare Diseases
Dermatology
Joints
Juvenile-onset scleroderma
Guidelines
Brain

Keywords

  • Diagnosis
  • Guideline
  • Localized scleroderma
  • Treatment

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Constantin, T., Foeldvari, I., Pain, C. E., Pálinkás, A., Höger, P., Moll, M., ... Torok, K. S. (Accepted/In press). Development of minimum standards of care for juvenile localized scleroderma. European Journal of Pediatrics, 1-17. https://doi.org/10.1007/s00431-018-3144-8

Development of minimum standards of care for juvenile localized scleroderma. / Constantin, T.; Foeldvari, Ivan; Pain, Clare E.; Pálinkás, Annamária; Höger, Peter; Moll, Monika; Nemkova, Dana; Weibel, Lisa; Laczkovszki, Melinda; Clements, Philip; Torok, Kathryn S.

In: European Journal of Pediatrics, 04.05.2018, p. 1-17.

Research output: Contribution to journalArticle

Constantin, T, Foeldvari, I, Pain, CE, Pálinkás, A, Höger, P, Moll, M, Nemkova, D, Weibel, L, Laczkovszki, M, Clements, P & Torok, KS 2018, 'Development of minimum standards of care for juvenile localized scleroderma', European Journal of Pediatrics, pp. 1-17. https://doi.org/10.1007/s00431-018-3144-8
Constantin, T. ; Foeldvari, Ivan ; Pain, Clare E. ; Pálinkás, Annamária ; Höger, Peter ; Moll, Monika ; Nemkova, Dana ; Weibel, Lisa ; Laczkovszki, Melinda ; Clements, Philip ; Torok, Kathryn S. / Development of minimum standards of care for juvenile localized scleroderma. In: European Journal of Pediatrics. 2018 ; pp. 1-17.
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