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- Open Access
Clinically inactive disease in juvenile dermatomyositis – a proposed revision to the printo criteria
© Almeida et al; licensee BioMed Central Ltd. 2014
- Published: 17 September 2014
- Skin Disease
- Data Entry
- Skin Rash
- Independent Cohort
Juvenile dermatomyositis (JDM) affects 3 children/million/year with myositis and skin disease being the typical features. PRINTO have recently established criteria to classify JDM patients who are clinically inactive by meeting at least 3 out of the following 4 conditions – CK ≤150, CMAS ≥48, MMT8 ≥78 and physician global VAS (PGA) ≤0.2. CK, CMAS and MMT8 all measure muscle involvement, only PGA includes skin or other organ involvement. The hypothesis that these criteria may fail to detect patients who have active skin disease but normal muscle parameters was tested.
To demonstrate the prevalence of clinically inactive disease in the UK JDM Cohort and Biomarker Study and to identify whether skin disease is still present in these patients on the basis of the PRINTO criteria.
Data were analysed from children who were recruited and met Bohan-Peter criteria. Data from patient episodes (either a clinic visit or hospital admission) were assessed using the PRINTO criteria. Using the PRINTO rules stipulating 3 of 4 criteria are required, all data entries were divided into 2 groups based on the criterion that was omitted. Each case was analysed to determine whether skin disease was present or absent.
Frequency of skin changes in JDM patients meeting PRINTO criteria (number of episodes, % in brackets)
Criteria met (DE=data entries, n=number of patients)
All 4 criteria met (255 DE, n 119)
3 criteria met, but PGA not met (320 DE, n 131)
3 criteria met of which one was PGA (107 DE, n 71)
This study is one of the first to test the PRINTO criteria in a large independent cohort of JDM patients. When clinically inactive disease is defined by “muscle-based” criteria, without PGA, there is a greater frequency of skin disease. As a revision, we propose that PGA should be included as an essential criterion together with 2 of the 3 muscle criteria. This would prevent skin disease being overlooked in the clinical assessment which is important since it is often recalcitrant to treatment and may be associated with poor long-term disease outcomes. A revision of the criteria would need testing in independent cohorts.
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.