Skip to main content
  • Poster presentation
  • Open access
  • Published:

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


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 at diagnosis incurs a worse outcome [1]. Most deaths occur early in the disease course [1, 2].


To develop recommendations for investigation of cardiopulmonary 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 cardiologist was invited. A nominal group technique was used. 75% consensus was defined as agreement.


Table 1 shows the recommendations for screening for cardiopulmonary 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 (in Table 1).

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

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


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


pulmonary function tests with diffusion capacity of lung for carbon monoxide


6 minute walk test

Author information

Authors and Affiliations



Rights and permissions

Open Access  This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made.

The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder.

To view a copy of this licence, visit

The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Pain, C., Constantin, T., Baildam, E. et al. Cardiopulmonary involvement in juvenile systemic sclerosis: development of recommendations for screening and investigation. Pediatr Rheumatol 12 (Suppl 1), P150 (2014).

Download citation

  • Published:

  • DOI: