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  • Poster presentation
  • Open Access

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

  • 1,
  • 2,
  • 1,
  • 3,
  • 4,
  • 5,
  • 6,
  • 7,
  • 8 and
Pediatric Rheumatology201412 (Suppl 1) :P52

https://doi.org/10.1186/1546-0096-12-S1-P52

  • Published:

Keywords

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

Introduction

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].

Objectives

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

Methods

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.

Results

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

Gastrointestinal

Baseline

 

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

 

Follow-up

 

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.

Conclusion

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.

Abbreviations

BP: 

blood pressure

ECG: 

electrocardiogram

ECHO: 

echocardiogram

MRI: 

magnetic resonance imaging

HRCT: 

high resolution computerised tomography

PFT: 

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

6MWT: 

6 minute walk test

Authors’ Affiliations

(1)
Pediatric Rheumatology, Liverpool, UK
(2)
Pediatric Rheumatology, Budapest, Hungary
(3)
Pediatric Cardiology, Hamburg, Germany
(4)
Pediatric Gastroenterology, Hamburg, Germany
(5)
Pediatric Rheumatology, Indiana, USA
(6)
Pediatric Rheumatology, Prague, Czech Republic
(7)
Pediatric Rheumatology, London, UK
(8)
Pediatric Rheumatology, Hamburg, Germany

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