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European Journal of Pediatrics

, Volume 177, Issue 7, pp 961–977 | Cite as

Development of minimum standards of care for juvenile localized scleroderma

  • Tamás ConstantinEmail author
  • Ivan Foeldvari
  • Clare E. Pain
  • Annamária Pálinkás
  • Peter Höger
  • Monika Moll
  • Dana Nemkova
  • Lisa Weibel
  • Melinda Laczkovszki
  • Philip Clements
  • Kathryn S. Torok
Review

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.

What is Known:

• Children with juvenile localized scleroderma (jLS) are managed by a number of specialties including pediatric rheumatologists and dermatologists, sometimes in shared clinics. Studies have shown that management varies considerably and that there are notable differences between specialties [1].

• There is very little published guidance on management of jLS.

What is new:

• These recommendations aim to standardize diagnosis, assessment, and management through review of pediatric evidence and consensus agreement.

• Joint review of patients by both pediatric rheumatologists and dermatologists is recommended.

Keywords

Localized scleroderma Diagnosis Treatment Guideline 

Abbreviations

AHA

Anti-histone antibodies

ANA

Antinuclear antibody

CARRA

Childhood Arthritis and Rheumatology Alliance

CDLQI

Children’s Dermatology Life Quality Index

CPK

Creatinine phosphokinase

CRP

C-reactive protein

DIET

Dyspigmentation, Induration, Erythema, Telengiectasias

DMARD

Disease-modifying anti-rheumatic drug

ECDS

En coup de saber

EEG

Electro-encephalogram

ESR

Erythrocyte sedimentation rate

EULAR

European League Against Rheumatism

JLS

Juvenile localized scleroderma

LOCUS

Localized Scleroderma Clinical and Ultrasound Study

LoSCAT

Localized Scleroderma Cutaneous Assessment Tool

LoSDI

Localized Scleroderma Damage Index

LS

Localized scleroderma

mLoSSI

Modified Localized Scleroderma Severity Skin Index

MMF

Mycophenolate mofetil

mRSS

Modified Rodnan Skin Score

MTX

Methotrexate

PRES

Pediatric Rheumatology European Society

PUVA therapy

Psoralen ultraviolet A

QoL

Quality of life

SSc

Systemic sclerosis

UV

Ultraviolet

Notes

Authors’ Contributions

Kathryn S. Torok is senior author. Ivan Foeldvari designed the initiative. Annamária Pálinkás, Melinda Laczkovszki performed the systematic literature review, supervised by Tamás Constantin. Validity assessment of selected papers was done by Tamás Constantin, Ivan Foeldvari, Clare E Pain, Peter Höger, Monika Moll, Dana Nemkova, Lisa Weibel, Kathryn S. Torok. Recommendations were formulated by Tamás Constantin, Ivan Foeldvari and Clare E Pain. The expert committee consisted of Tamás Constantin, Ivan Foeldvari, Clare E Pain, Peter Höger, Monika Moll, Dana Nemkova, Lisa Weibel, Kathryn S. Torok; they completed the online surveys and participated in consensus meeting. Philip Clements assisted in the preparation of the live consensus meeting and facilitated the consensus procedure using nominal group technique. Tamás Constantin, Clare E Pain and Kathryn S. Torok wrote the manuscript, with contribution and approval of all co-authors.

Informed consent

no confidential data were used regarding this work.

Supplementary material

431_2018_3144_MOESM1_ESM.docx (40 kb)
ESM 1 (39.5 kb)

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Tamás Constantin
    • 1
    Email author
  • Ivan Foeldvari
    • 2
  • Clare E. Pain
    • 3
  • Annamária Pálinkás
    • 1
  • Peter Höger
    • 4
  • Monika Moll
    • 5
  • Dana Nemkova
    • 6
  • Lisa Weibel
    • 7
  • Melinda Laczkovszki
    • 1
  • Philip Clements
    • 8
  • Kathryn S. Torok
    • 9
  1. 1.Semmelweis UniversityBudapestHungary
  2. 2.Hamburger Zentrum für Kinder- und JugendrheumatologieHamburgGermany
  3. 3.Alder Hey Children’s NHS Foundation TrustLiverpoolUK
  4. 4.Department of Paediatric DermatologyCatholic Children’s Hospital WilhelmstiftHamburgGermany
  5. 5.University Childrens HospitalTuebingenGermany
  6. 6.Charles UniversityPragueCzech Republic
  7. 7.Department of DermatologyUniversity Children’s Hospital ZurichZurichSwitzerland
  8. 8.David Geffen School of Medicine at UCLA, Division of RheumatologyLos AngelesUSA
  9. 9.University of PittsburghPittsburghUSA

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