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  • Open Access

Gastrointestinal involvement in juvenile systemic sclerosis: development of recommendations for screening and investigation

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Pediatric Rheumatology201412 (Suppl 1) :P52

  • Published:


  • Screening Program
  • Rare Disease
  • Scleroderma
  • Consensus Expert
  • Significant Mortality


There are currently no agreed recommendations on how to investigate children for gastrointestinal (GI) involvement in Juvenile Systemic Sclerosis (JSSc). The aim of screening is to detect disease early to facilitate early aggressive therapy and improve outcomes. GI involvement at diagnosis incurs a worse outcome [1]. Most deaths occur early in the disease course [1, 2].


To develop recommendations for investigation of GI involvement in JSSc, based on paediatric evidence and where this was lacking, consensus expert agreement.


Members of the PRES Scleroderma Working Group were invited to participate; additionally a paediatric gastroenterologist was invited. A nominal group technique was used. 75% consensus was defined as agreement.


Table 1 shows the recommendations for screening for GI involvement at baseline and at defined time points from diagnosis. Other recommendations agreed by the group which are relevant at any stage in the disease course are as follows:

Table 1




All patients should have a barium swallow to assess for dysmotility or stricture and 24 hour pH monitoring for GORD and progress to upper GI endoscopy if any abnormality detected




Every 3 years or sooner if worsening lung involvement and/or worsening GI symptoms

Upper GI endoscopy

Barium swallow

24 hours pH monitoring

*screening guidelines are based on asymptomatic patients. However, children may need more frequent monitoring depending on clinical status and abnormalities detected on previous investigation.


JSSc has a significant mortality particularly early on in the disease course. The objective of an aggressive screening program is to identify GI involvement at a stage which may be amenable to treatment. The recommendations developed by this group aim to standardise care and improve outcomes in this rare disease.

Disclosure of interest

None declared.



blood pressure






magnetic resonance imaging


high resolution computerised tomography


DLCO pulmonary function tests with diffusion capacity of lung for carbon monoxide


6 minute walk test

Authors’ Affiliations

Pediatric Rheumatology, Liverpool, UK
Pediatric Rheumatology, Budapest, Hungary
Pediatric Cardiology, Hamburg, Germany
Pediatric Gastroenterology, Hamburg, Germany
Pediatric Rheumatology, Indiana, USA
Pediatric Rheumatology, Prague, Czech Republic
Pediatric Rheumatology, London, UK
Pediatric Rheumatology, Hamburg, Germany


© Pain et al; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.