ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1325

Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE): Evidence Based Recommendations for Diagnosis and Treatment of Juvenile Localized Scleroderma and Juvenile Systemic Sclerosis

Bas Vastert1, Roberta Culpo2, Jordi Anton3, Tadej Avcin4, Eileen Baildam5, Christina Boros6, Tamás Constantin7, Jeff Chaitow8, Pavla Dolezalova9, Ozgur Kasapcopur10, Sheila Oliveira11, Clarissa Pilkington12, Annet van Royen-Kerkhof13, Ricardo A. G. Russo14, Claudia Saad-Magalhaes15, Natasa Toplak16, Angelo Ravelli17, Nico Wulffraat18, Ivan Foeldvari19 and Francesco Zulian20, 1University Medical Center Utrecht, Utrecht, Netherlands, 2Department of Pediatrics., University of Padua, Padua, Italy, 3Pediatric Rheumatology Unit. Hospital Sant Joan de Déu. Universitat de Barcelona, Barcelona, Spain, 4Pediatric Rheumatology, University Children´s Hospital, Ljubjana, Slovenia, 5Paediatric Rheumatology, Alder Hey Children's Foundation NHS Trust, Liverpool, United Kingdom, 6University of Adelaide, Adelaide, Australia, 7Pediatric Rheumatology, University Childrens Hospital, Budapest, Hungary, 8The Children’s Hospital Westmead, Sydney, Australia, 9Department of Paediatrics and Adolescent Medicine, Charles University, Prague, Czech Republic, 10University Cerrahpasa Faculty of Medicine, Istanbul, Turkey, 11Pediatric Rheumatology, Universidade F Rio De Janeiro, Rio De Janeiro, Brazil, 12Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 13Paediatric Immunology and Rheumatology, University Medical Centre Utrecht - Wilhelmina Children's Hospital, Utrecht, Netherlands, 14Immunology & Rheumatology, Hospital de Pediatria Garrahan, Buenos Aires, Argentina, 15Faculdade de Medicina de Botucatu, Botucatu, Brazil, 16Pediatric Rheumatology, University Medical Center, Ljubliana, Slovenia, 17Istituto Giannina Gaslini and University of Genova, Genova, Italy, 18Pediatric rheumatology, Wilhelmina Children's Hospital/ UMC Utrecht, Utrecht, Netherlands, 19Department of Pediatric Rheumatology, Hamburger Zentrum für Kinder und Jugendrheumatologie, Hamburg, Germany, 20Pediatrics/Rheumatology Div, University of Padua, Padua, Italy

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: , , ,

Session Information

Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects: Pediatric Lupus, Scleroderma and Myositis (ACR)

Session Type: Abstract Submissions (ACR)

Background/Purpose

Juvenile Localized Scleroderma (JLS) and Juvenile Systemic Sclerosis (JSSc) form a group of rare pediatric diseases that can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physician’s experience. In 2012, a European initiative called SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Purpose:  to provide evidence based recommendations for diagnosis and treatment of Juvenile Scleroderma.

Methods

Evidence based recommendations were developed using the European League Against Rheumatism (EULAR) standard operating procedure. An expert committee was instituted, consisting of pediatric rheumatologists and experts in Juvenile Scleroderma. The expert committee defined search terms for the systematic literature review. Two independent experts scored articles for validity and level of evidence. Recommendations derived from the literature were evaluated by an online survey. Those with less than 80% agreement during the online survey were reformulated. Subsequently, all recommendations were discussed at a consensus meeting using the nominal group technique. Recommendations were accepted if more than 80% agreement was reached.

Results

The literature search yielded 1550 articles for JLS and 8562 for JSSc, of which 52 and 37, respectively (25 for diagnosis and 27 for treatment of JLS, 21 for diagnosis and 16 for treatment of JSSc) were considered relevant and therefore scored for validity and level of evidence. 42 articles (15 for diagnosis and 14 for treatment of JLS, 6 for diagnosis and 7 for treatment of JSSc) were scored valid and used in the formulation of the recommendations. Eleven recommendations for diagnosis and 7 for treatment were suggested in the online survey on JLS. Ten recommendations for diagnosis and 6 for treatment were accepted with more than 80% agreement after the consensus meeting. Six recommendations for diagnosis and 5 for treatment were suggested in the online survey on JSSc. Six recommendations for diagnosis and 4 for treatment were accepted with more than 80% agreement after the consensus meeting. Topics covered for diagnosis and for treatment are showed in Table 1.

JLS

DIAGNOSIS

TREATMENT

Disease activity assessment and response to therapy

Clinical scores

Topical treatment

Medium-dose UVA1 phototherapy

Thermography

Imiquimod

Ultrasounds

Systemic treatment

Corticosteroids

Disease severity and damage assessment

Clinical scores

Metotrexate

Extra-cutaneous involvement (articular, musculoskeletal, neurological, ophtalmological, dental, maxillo-facial)

Clinical assessment

Mychophenolate mofetil

Musculoskeletal MRI

Brain MRI

JSSc

DIAGNOSIS

TREATMENT

Disease severity and damage assessment

Clinical scores

Skin and lung involvement

Mychophenolate mofetil

Skin involvement

Clinical scores

Vascular involvement

Bosentan

Lung involvement

Pulmonary function tests

Progressive and refractory disease

Autologous stem-cell transplantation

Serological markers

Raynaud’s phenomenon

Capillaroscopy

Ineffective treatments

D-penicillamine

Conclusion

The SHARE initiative provides recommendations for diagnosis and treatment for Juvenile Scleroderma and thereby facilitates improvement and uniformity of care throughout Europe.

Disclosure:

B. Vastert,
None;

R. Culpo,
None;

J. Anton,
None;

T. Avcin,
None;

E. Baildam,
None;

C. Boros,
None;

T. Constantin,
None;

J. Chaitow,
None;

P. Dolezalova,
None;

O. Kasapcopur,
None;

S. Oliveira,
None;

C. Pilkington,
None;

A. van Royen-Kerkhof,
None;

R. A. G. Russo,
None;

C. Saad-Magalhaes,
None;

N. Toplak,
None;

A. Ravelli,

None,

8;

N. Wulffraat,
None;

I. Foeldvari,

Novartis Pharma AG, Abbott, Chugai, Genzyme,

5;

F. Zulian,
None.

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