Background/Purpose

There are currently no agreed recommendations on how to investigate children for cardiopulmonary involvement in Juvenile Systemic Sclerosis (JSSc). The aim of screening is to detect disease early to facilitate early aggressive therapy and improve outcomes. Cardiopulmonary involvement is the leading cause of death in JSSc and cardiopulmonary 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 cardiopulmonary 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 were invited. A nominal group technique was used. 75% consensus was defined as agreement.

Results

ResultsTable 1 shows the recommendations for screening for cardiopulmonary and 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. If there are any concerns or signs of pulmonary hypertension then right heart catheterisation should be undertaken.
2. Any child with exertional chest pain or abnormality on 24hr ECG should undergo exercise ECG (if old enough to comply).
3. Those with worsening PFTS or clinical deterioration should have HRCT thorax repeated sooner (particularly, FVC <70% or DLCO <80% or drop in values by 20% of baseline).

Recommendations are based on low grade evidence and in the most part from expert consensus opinion with extrapolation from adult studies

Table 1. Recommendations for screening for cardiopulmonary and GI involvement in JSSc at baseline and follow-up (75% consensus defined as agreement).

Cardiopulmonary	Baseline
	All patients should undergo: –        BP –        12 lead ECG –        24 hour ECG –        ECHO with Doppler –        Cardiac MRI with gadolinium –        HRCT thorax –        PFT with DLCO –        6MWT
	Follow-up screening (for first 5 years from diagnosis)*
	6 monthly	12 lead ECG ECHO with doppler 6MWT PFT with DLCO
	Annual	24hr ECG
	At 3 years	Repeat HRCT

Conclusion

JSSc has a significant mortality particularly early on in the disease course. The objective of an aggressive screening program is to identify cardiopulmonary and 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.

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