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

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 Table 1

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

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Correspondence to Ivan Foeldvari.

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Pain, C., Constantin, T., Baildem, E. et al. Gastrointestinal involvement in juvenile systemic sclerosis: development of recommendations for screening and investigation. Pediatr Rheumatol 12, P52 (2014). https://doi.org/10.1186/1546-0096-12-S1-P52

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Keywords

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