Background/Purpose

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 cardiologist and paediatric gastroenterologist were 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:

1. Oesophageal dilatation should be assessed on any HRCT thorax performed.
2. If any concerns regarding bleeding such as chronic anaemia, consider upper GI endoscopy.
3. Any patient with nausea, vomiting, abdominal pain, bloating, diarrhoea or poor weight gain should undergo a hydrogen breath test for bacterial overgrowth.
4. Consider dietetic or gastroenterology review to assess nutritional intake and status.
5. Any patient with significant persistent GI symptoms including poor weight gain should be referred to a paediatric gastroenterologist.
6. 6.    Table 1. Recommendations for screening for GI involvement in JSSc at baseline and follow-up (75% consensus defined as agreement).

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 

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.

1.             Martini G, Vittadello F, Kasapcopur O, Magni Manzoni S, Corona F, Duarte-Salazar C, et al. Factors affecting survival in juvenile systemic sclerosis. Rheumatology (Oxford). 2009;48(2):119-22.

2.             Foeldvari I, Zhavania M, Birdi N, Cuttica RJ, de Oliveira SH, Dent PB, et al. Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey. Rheumatology (Oxford). 2000;39(5):556-9.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/gastrointestinal-involvement-in-juvenile-systemic-sclerosis-development-of-recommendations-for-screening-and-investigation/