Cardiopulmonary involvement in juvenile systemic sclerosis: development of recommendations for screening and investigation
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KeywordsScleroderma Consensus Expert Paediatric Cardiologist Adult Study Aggressive Therapy
magnetic resonance imaging
high resolution computerised tomography
- PFT DLCO
pulmonary function tests with diffusion capacity of lung for carbon monoxide
6 minute walk test
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 . 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.
Recommendations for screening for cardiopulmonary involvement in JSSc at baseline and follow-up (75% consensus defined as agreement)
All patients should undergo:
- 12 lead ECG
- 24 hour ECG
- ECHO with Doppler
- Cardiac MRI with gadolinium
- HRCT thorax
- PFT with DLCO
Follow-up screening (for first 5 years from diagnosis)*
12 lead ECG
ECHO with doppler
PFT with DLCO
At 3 years
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
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.